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Hypnosis May Offer Relief During Sharp Debridement of Skin Ulcers
TOPLINE:
Hypnosis reduces pain during sharp debridement of skin ulcers in patients with immune-mediated inflammatory diseases, with most patients reporting decreased pain awareness and lasting pain relief for 2-3 days after the procedure.
METHODOLOGY:
- Researchers reported their experience with the anecdotal use of hypnosis for pain management in debridement of skin ulcers in immune-mediated inflammatory diseases.
- They studied 16 participants (14 women; mean age, 56 years; 14 with systemic sclerosis or morphea) with recurrent skin ulcerations requiring sharp debridement, who presented to a wound care clinic at the Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. The participants had negative experiences with pharmacologic pain management.
- Participants consented to hypnosis during debridement as the only mode of analgesia, conducted by the same hypnosis-trained, experienced healthcare professional in charge of their ulcer care.
- Ulcer pain scores were recorded using a numerical rating pain scale before and immediately after debridement, with a score of 0 indicating no pain and 10 indicating worst pain.
TAKEAWAY:
- Hypnosis reduced the median pre-debridement ulcer pain score from 8 (interquartile range [IQR], 7-10) to 0.5 (IQR, 0-2) immediately after the procedure.
- Of 16 participants, 14 reported being aware of the procedure but not feeling the pain, with only two participants experiencing a brief spike in pain.
- The other two participants reported experiencing reduced awareness and being pain-free during the procedure.
- Five participants reported a lasting decrease in pain perception for 2-3 days after the procedure.
IN PRACTICE:
“These preliminary data underscore the potential for the integration of hypnosis into the management of intervention-related pain in clinical care,” the authors wrote.
SOURCE:
The study was led by Begonya Alcacer-Pitarch, PhD, Leeds Institute of Rheumatic and Musculoskeletal Medicine, the University of Leeds, and Chapel Allerton Hospital in Leeds, United Kingdom. It was published as a correspondence on September 10, 2024, in The Lancet Rheumatology.
LIMITATIONS:
The small sample size may limit the generalizability of the findings. The methods used for data collection were not standardized, and the individuals included in the study may have introduced selection bias.
DISCLOSURES:
The study did not have a funding source. The authors declared no relevant conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Hypnosis reduces pain during sharp debridement of skin ulcers in patients with immune-mediated inflammatory diseases, with most patients reporting decreased pain awareness and lasting pain relief for 2-3 days after the procedure.
METHODOLOGY:
- Researchers reported their experience with the anecdotal use of hypnosis for pain management in debridement of skin ulcers in immune-mediated inflammatory diseases.
- They studied 16 participants (14 women; mean age, 56 years; 14 with systemic sclerosis or morphea) with recurrent skin ulcerations requiring sharp debridement, who presented to a wound care clinic at the Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. The participants had negative experiences with pharmacologic pain management.
- Participants consented to hypnosis during debridement as the only mode of analgesia, conducted by the same hypnosis-trained, experienced healthcare professional in charge of their ulcer care.
- Ulcer pain scores were recorded using a numerical rating pain scale before and immediately after debridement, with a score of 0 indicating no pain and 10 indicating worst pain.
TAKEAWAY:
- Hypnosis reduced the median pre-debridement ulcer pain score from 8 (interquartile range [IQR], 7-10) to 0.5 (IQR, 0-2) immediately after the procedure.
- Of 16 participants, 14 reported being aware of the procedure but not feeling the pain, with only two participants experiencing a brief spike in pain.
- The other two participants reported experiencing reduced awareness and being pain-free during the procedure.
- Five participants reported a lasting decrease in pain perception for 2-3 days after the procedure.
IN PRACTICE:
“These preliminary data underscore the potential for the integration of hypnosis into the management of intervention-related pain in clinical care,” the authors wrote.
SOURCE:
The study was led by Begonya Alcacer-Pitarch, PhD, Leeds Institute of Rheumatic and Musculoskeletal Medicine, the University of Leeds, and Chapel Allerton Hospital in Leeds, United Kingdom. It was published as a correspondence on September 10, 2024, in The Lancet Rheumatology.
LIMITATIONS:
The small sample size may limit the generalizability of the findings. The methods used for data collection were not standardized, and the individuals included in the study may have introduced selection bias.
DISCLOSURES:
The study did not have a funding source. The authors declared no relevant conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Hypnosis reduces pain during sharp debridement of skin ulcers in patients with immune-mediated inflammatory diseases, with most patients reporting decreased pain awareness and lasting pain relief for 2-3 days after the procedure.
METHODOLOGY:
- Researchers reported their experience with the anecdotal use of hypnosis for pain management in debridement of skin ulcers in immune-mediated inflammatory diseases.
- They studied 16 participants (14 women; mean age, 56 years; 14 with systemic sclerosis or morphea) with recurrent skin ulcerations requiring sharp debridement, who presented to a wound care clinic at the Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom. The participants had negative experiences with pharmacologic pain management.
- Participants consented to hypnosis during debridement as the only mode of analgesia, conducted by the same hypnosis-trained, experienced healthcare professional in charge of their ulcer care.
- Ulcer pain scores were recorded using a numerical rating pain scale before and immediately after debridement, with a score of 0 indicating no pain and 10 indicating worst pain.
TAKEAWAY:
- Hypnosis reduced the median pre-debridement ulcer pain score from 8 (interquartile range [IQR], 7-10) to 0.5 (IQR, 0-2) immediately after the procedure.
- Of 16 participants, 14 reported being aware of the procedure but not feeling the pain, with only two participants experiencing a brief spike in pain.
- The other two participants reported experiencing reduced awareness and being pain-free during the procedure.
- Five participants reported a lasting decrease in pain perception for 2-3 days after the procedure.
IN PRACTICE:
“These preliminary data underscore the potential for the integration of hypnosis into the management of intervention-related pain in clinical care,” the authors wrote.
SOURCE:
The study was led by Begonya Alcacer-Pitarch, PhD, Leeds Institute of Rheumatic and Musculoskeletal Medicine, the University of Leeds, and Chapel Allerton Hospital in Leeds, United Kingdom. It was published as a correspondence on September 10, 2024, in The Lancet Rheumatology.
LIMITATIONS:
The small sample size may limit the generalizability of the findings. The methods used for data collection were not standardized, and the individuals included in the study may have introduced selection bias.
DISCLOSURES:
The study did not have a funding source. The authors declared no relevant conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
Belimumab Hits Newer Remission, Low Disease Activity Metrics
TOPLINE:
A greater proportion of patients with active systemic lupus erythematosus (SLE) treated with belimumab plus standard therapy achieved the newest definitions for remission and low disease activity compared with those treated with placebo plus standard therapy, with benefits observed as early as week 28 for remission and week 8 for disease activity, according to pooled results from five clinical trials.
METHODOLOGY:
- Researchers conducted an integrated post hoc analysis of five randomized phase 3 clinical trials to evaluate the attainment of remission and low disease activity in adult patients with active, autoantibody-positive SLE.
- A total of 3086 patients (median age, 36 years; 94% women) were randomly assigned to receive standard therapy with intravenous belimumab 10 mg/kg monthly or subcutaneous belimumab 200 mg weekly (n = 1869) or placebo (n = 1217).
- The proportion of patients who achieved definitions of remission in SLE (DORIS) remission and lupus low disease activity state (LLDAS) by visit up to week 52 was assessed.
- The analysis also evaluated the time taken to achieve sustained (at least two consecutive visits) and maintained (up to week 52) DORIS remission and LLDAS.
TAKEAWAY:
- At week 52, a higher proportion of patients receiving belimumab vs placebo achieved DORIS remission (8% vs 6%; risk ratio [RR], 1.51; P = .0055) and LLDAS (17% vs 10%; RR, 1.74; P < .0001).
- The earliest observed significant benefit of belimumab over placebo in patients with a higher baseline disease activity was at week 20 for DORIS remission (RR, 2.09; P = .043) and at week 16 for LLDAS (RR, 1.46; P = .034), with both maintained through week 52.
- The proportion of patients who attained DORIS remission and LLDAS as early as week 28 and week 8, respectively, was higher in the belimumab group than in the placebo group, with both maintained through week 52.
- Patients on belimumab were more likely to have a sustained and maintained DORIS remission (hazard ratio [HR], 1.53; P = .013) and LLDAS (HR, 1.79; P < .0001) at any timepoint.
IN PRACTICE:
“The data clearly support that belimumab is a valuable addition toward accomplishing and maintaining remission or LLDAS,” George Bertsias, MD, PhD, University of Crete Medical School, Heraklion, Greece, and Jinoos Yazdany, MD, University of California San Francisco, wrote in a related comment.
SOURCE:
This study, led by Ioannis Parodis, MD, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, was published online on August 26, 2024, in The Lancet Rheumatology.
LIMITATIONS:
Due to the post hoc nature of the analysis, the trials were not specifically designed to have adequate statistical power to demonstrate the difference between patients who did or did not achieve DORIS remission or LLDAS. The analysis was limited to patients who met the eligibility criteria, and the outcomes are not generalizable to populations outside a clinical trial setting. The study population had high disease activity, which made it challenging to attain the treatment targets.
DISCLOSURES:
The five trials included in this analysis were funded by GSK. The study was supported by the Swedish Rheumatism Association, King Gustaf V’s 80-year Foundation, the Swedish Society of Medicine, Nyckelfonden, Professor Nanna Svartz Foundation, Ulla and Roland Gustafsson Foundation, Region Stockholm, and Karolinska Institutet. Some authors reported receiving grants, speaker honoraria, or consulting fees from various pharmaceutical companies. Some authors reported being employees and owning stocks and shares of GSK.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
A greater proportion of patients with active systemic lupus erythematosus (SLE) treated with belimumab plus standard therapy achieved the newest definitions for remission and low disease activity compared with those treated with placebo plus standard therapy, with benefits observed as early as week 28 for remission and week 8 for disease activity, according to pooled results from five clinical trials.
METHODOLOGY:
- Researchers conducted an integrated post hoc analysis of five randomized phase 3 clinical trials to evaluate the attainment of remission and low disease activity in adult patients with active, autoantibody-positive SLE.
- A total of 3086 patients (median age, 36 years; 94% women) were randomly assigned to receive standard therapy with intravenous belimumab 10 mg/kg monthly or subcutaneous belimumab 200 mg weekly (n = 1869) or placebo (n = 1217).
- The proportion of patients who achieved definitions of remission in SLE (DORIS) remission and lupus low disease activity state (LLDAS) by visit up to week 52 was assessed.
- The analysis also evaluated the time taken to achieve sustained (at least two consecutive visits) and maintained (up to week 52) DORIS remission and LLDAS.
TAKEAWAY:
- At week 52, a higher proportion of patients receiving belimumab vs placebo achieved DORIS remission (8% vs 6%; risk ratio [RR], 1.51; P = .0055) and LLDAS (17% vs 10%; RR, 1.74; P < .0001).
- The earliest observed significant benefit of belimumab over placebo in patients with a higher baseline disease activity was at week 20 for DORIS remission (RR, 2.09; P = .043) and at week 16 for LLDAS (RR, 1.46; P = .034), with both maintained through week 52.
- The proportion of patients who attained DORIS remission and LLDAS as early as week 28 and week 8, respectively, was higher in the belimumab group than in the placebo group, with both maintained through week 52.
- Patients on belimumab were more likely to have a sustained and maintained DORIS remission (hazard ratio [HR], 1.53; P = .013) and LLDAS (HR, 1.79; P < .0001) at any timepoint.
IN PRACTICE:
“The data clearly support that belimumab is a valuable addition toward accomplishing and maintaining remission or LLDAS,” George Bertsias, MD, PhD, University of Crete Medical School, Heraklion, Greece, and Jinoos Yazdany, MD, University of California San Francisco, wrote in a related comment.
SOURCE:
This study, led by Ioannis Parodis, MD, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, was published online on August 26, 2024, in The Lancet Rheumatology.
LIMITATIONS:
Due to the post hoc nature of the analysis, the trials were not specifically designed to have adequate statistical power to demonstrate the difference between patients who did or did not achieve DORIS remission or LLDAS. The analysis was limited to patients who met the eligibility criteria, and the outcomes are not generalizable to populations outside a clinical trial setting. The study population had high disease activity, which made it challenging to attain the treatment targets.
DISCLOSURES:
The five trials included in this analysis were funded by GSK. The study was supported by the Swedish Rheumatism Association, King Gustaf V’s 80-year Foundation, the Swedish Society of Medicine, Nyckelfonden, Professor Nanna Svartz Foundation, Ulla and Roland Gustafsson Foundation, Region Stockholm, and Karolinska Institutet. Some authors reported receiving grants, speaker honoraria, or consulting fees from various pharmaceutical companies. Some authors reported being employees and owning stocks and shares of GSK.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
A greater proportion of patients with active systemic lupus erythematosus (SLE) treated with belimumab plus standard therapy achieved the newest definitions for remission and low disease activity compared with those treated with placebo plus standard therapy, with benefits observed as early as week 28 for remission and week 8 for disease activity, according to pooled results from five clinical trials.
METHODOLOGY:
- Researchers conducted an integrated post hoc analysis of five randomized phase 3 clinical trials to evaluate the attainment of remission and low disease activity in adult patients with active, autoantibody-positive SLE.
- A total of 3086 patients (median age, 36 years; 94% women) were randomly assigned to receive standard therapy with intravenous belimumab 10 mg/kg monthly or subcutaneous belimumab 200 mg weekly (n = 1869) or placebo (n = 1217).
- The proportion of patients who achieved definitions of remission in SLE (DORIS) remission and lupus low disease activity state (LLDAS) by visit up to week 52 was assessed.
- The analysis also evaluated the time taken to achieve sustained (at least two consecutive visits) and maintained (up to week 52) DORIS remission and LLDAS.
TAKEAWAY:
- At week 52, a higher proportion of patients receiving belimumab vs placebo achieved DORIS remission (8% vs 6%; risk ratio [RR], 1.51; P = .0055) and LLDAS (17% vs 10%; RR, 1.74; P < .0001).
- The earliest observed significant benefit of belimumab over placebo in patients with a higher baseline disease activity was at week 20 for DORIS remission (RR, 2.09; P = .043) and at week 16 for LLDAS (RR, 1.46; P = .034), with both maintained through week 52.
- The proportion of patients who attained DORIS remission and LLDAS as early as week 28 and week 8, respectively, was higher in the belimumab group than in the placebo group, with both maintained through week 52.
- Patients on belimumab were more likely to have a sustained and maintained DORIS remission (hazard ratio [HR], 1.53; P = .013) and LLDAS (HR, 1.79; P < .0001) at any timepoint.
IN PRACTICE:
“The data clearly support that belimumab is a valuable addition toward accomplishing and maintaining remission or LLDAS,” George Bertsias, MD, PhD, University of Crete Medical School, Heraklion, Greece, and Jinoos Yazdany, MD, University of California San Francisco, wrote in a related comment.
SOURCE:
This study, led by Ioannis Parodis, MD, Karolinska Institutet and Karolinska University Hospital, Stockholm, Sweden, was published online on August 26, 2024, in The Lancet Rheumatology.
LIMITATIONS:
Due to the post hoc nature of the analysis, the trials were not specifically designed to have adequate statistical power to demonstrate the difference between patients who did or did not achieve DORIS remission or LLDAS. The analysis was limited to patients who met the eligibility criteria, and the outcomes are not generalizable to populations outside a clinical trial setting. The study population had high disease activity, which made it challenging to attain the treatment targets.
DISCLOSURES:
The five trials included in this analysis were funded by GSK. The study was supported by the Swedish Rheumatism Association, King Gustaf V’s 80-year Foundation, the Swedish Society of Medicine, Nyckelfonden, Professor Nanna Svartz Foundation, Ulla and Roland Gustafsson Foundation, Region Stockholm, and Karolinska Institutet. Some authors reported receiving grants, speaker honoraria, or consulting fees from various pharmaceutical companies. Some authors reported being employees and owning stocks and shares of GSK.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
Current Hydroxychloroquine Use in Lupus May Provide Protection Against Cardiovascular Events
TOPLINE:
Current use of hydroxychloroquine is associated with a lower risk for myocardial infarction (MI), stroke, and other thromboembolic events in patients with systemic lupus erythematosus (SLE). This protective effect diminishes after discontinuation of hydroxychloroquine treatment.
METHODOLOGY:
- Researchers used a nested case-control design to evaluate the association between exposure to hydroxychloroquine and the risk for cardiovascular events in patients with SLE.
- They included 52,883 adults with SLE (mean age, 44.23 years; 86.6% women) identified from the National System of Health Databases, which includes 99% of the French population.
- Among these, 1981 individuals with composite cardiovascular conditions were matched with 16,892 control individuals without cardiovascular conditions.
- Patients were categorized on the basis of hydroxychloroquine exposure into current users (last exposure within 90 days before a cardiovascular event), remote users (91-365 days before), and nonusers (no exposure within 365 days).
- The study outcomes included a composite of cardiovascular events, including MI, stroke (including transient ischemic attack), and other thromboembolic events such as phlebitis, thrombophlebitis, venous thrombosis, venous thromboembolism, and pulmonary embolism.
TAKEAWAY:
- Current hydroxychloroquine users had lower odds of experiencing a composite cardiovascular outcome than nonusers (adjusted odds ratio [aOR], 0.63; 95% CI, 0.57-0.70).
- The odds of MI (aOR, 0.72; 95% CI, 0.60-0.87), stroke (aOR, 0.71; 95% CI, 0.61-0.83), and other thromboembolic events (aOR, 0.58; 95% CI, 0.48-0.69) were also lower among current users than among nonusers.
- No significant association was found for remote hydroxychloroquine exposure and the risk for composite cardiovascular events, MI, stroke, and other thromboembolic events.
IN PRACTICE:
“These findings support the protective association of hydroxychloroquine against CV [cardiovascular] events and underscore the importance of continuous hydroxychloroquine therapy for patients diagnosed with SLE,” the authors wrote.
SOURCE:
The study was led by Lamiae Grimaldi-Bensouda, PharmD, PhD, Department of Pharmacology, Hospital Group Paris-Saclay, Assistance Publique-Hôpitaux de Paris, France. It was published online on August 30, 2024, in JAMA Network Open.
LIMITATIONS:
The observational nature of the study may have introduced confounding. Current hydroxychloroquine users were younger than nonusers, with an average age difference of almost 5 years. Current hydroxychloroquine users had a twofold longer duration of onset of SLE and had a higher prevalence of chronic kidney disease compared with nonusers.
DISCLOSURES:
This study was funded by the Banque pour l’Investissement, Deeptech. Some authors declared having financial ties with various institutions and companies outside of the current study.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Current use of hydroxychloroquine is associated with a lower risk for myocardial infarction (MI), stroke, and other thromboembolic events in patients with systemic lupus erythematosus (SLE). This protective effect diminishes after discontinuation of hydroxychloroquine treatment.
METHODOLOGY:
- Researchers used a nested case-control design to evaluate the association between exposure to hydroxychloroquine and the risk for cardiovascular events in patients with SLE.
- They included 52,883 adults with SLE (mean age, 44.23 years; 86.6% women) identified from the National System of Health Databases, which includes 99% of the French population.
- Among these, 1981 individuals with composite cardiovascular conditions were matched with 16,892 control individuals without cardiovascular conditions.
- Patients were categorized on the basis of hydroxychloroquine exposure into current users (last exposure within 90 days before a cardiovascular event), remote users (91-365 days before), and nonusers (no exposure within 365 days).
- The study outcomes included a composite of cardiovascular events, including MI, stroke (including transient ischemic attack), and other thromboembolic events such as phlebitis, thrombophlebitis, venous thrombosis, venous thromboembolism, and pulmonary embolism.
TAKEAWAY:
- Current hydroxychloroquine users had lower odds of experiencing a composite cardiovascular outcome than nonusers (adjusted odds ratio [aOR], 0.63; 95% CI, 0.57-0.70).
- The odds of MI (aOR, 0.72; 95% CI, 0.60-0.87), stroke (aOR, 0.71; 95% CI, 0.61-0.83), and other thromboembolic events (aOR, 0.58; 95% CI, 0.48-0.69) were also lower among current users than among nonusers.
- No significant association was found for remote hydroxychloroquine exposure and the risk for composite cardiovascular events, MI, stroke, and other thromboembolic events.
IN PRACTICE:
“These findings support the protective association of hydroxychloroquine against CV [cardiovascular] events and underscore the importance of continuous hydroxychloroquine therapy for patients diagnosed with SLE,” the authors wrote.
SOURCE:
The study was led by Lamiae Grimaldi-Bensouda, PharmD, PhD, Department of Pharmacology, Hospital Group Paris-Saclay, Assistance Publique-Hôpitaux de Paris, France. It was published online on August 30, 2024, in JAMA Network Open.
LIMITATIONS:
The observational nature of the study may have introduced confounding. Current hydroxychloroquine users were younger than nonusers, with an average age difference of almost 5 years. Current hydroxychloroquine users had a twofold longer duration of onset of SLE and had a higher prevalence of chronic kidney disease compared with nonusers.
DISCLOSURES:
This study was funded by the Banque pour l’Investissement, Deeptech. Some authors declared having financial ties with various institutions and companies outside of the current study.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Current use of hydroxychloroquine is associated with a lower risk for myocardial infarction (MI), stroke, and other thromboembolic events in patients with systemic lupus erythematosus (SLE). This protective effect diminishes after discontinuation of hydroxychloroquine treatment.
METHODOLOGY:
- Researchers used a nested case-control design to evaluate the association between exposure to hydroxychloroquine and the risk for cardiovascular events in patients with SLE.
- They included 52,883 adults with SLE (mean age, 44.23 years; 86.6% women) identified from the National System of Health Databases, which includes 99% of the French population.
- Among these, 1981 individuals with composite cardiovascular conditions were matched with 16,892 control individuals without cardiovascular conditions.
- Patients were categorized on the basis of hydroxychloroquine exposure into current users (last exposure within 90 days before a cardiovascular event), remote users (91-365 days before), and nonusers (no exposure within 365 days).
- The study outcomes included a composite of cardiovascular events, including MI, stroke (including transient ischemic attack), and other thromboembolic events such as phlebitis, thrombophlebitis, venous thrombosis, venous thromboembolism, and pulmonary embolism.
TAKEAWAY:
- Current hydroxychloroquine users had lower odds of experiencing a composite cardiovascular outcome than nonusers (adjusted odds ratio [aOR], 0.63; 95% CI, 0.57-0.70).
- The odds of MI (aOR, 0.72; 95% CI, 0.60-0.87), stroke (aOR, 0.71; 95% CI, 0.61-0.83), and other thromboembolic events (aOR, 0.58; 95% CI, 0.48-0.69) were also lower among current users than among nonusers.
- No significant association was found for remote hydroxychloroquine exposure and the risk for composite cardiovascular events, MI, stroke, and other thromboembolic events.
IN PRACTICE:
“These findings support the protective association of hydroxychloroquine against CV [cardiovascular] events and underscore the importance of continuous hydroxychloroquine therapy for patients diagnosed with SLE,” the authors wrote.
SOURCE:
The study was led by Lamiae Grimaldi-Bensouda, PharmD, PhD, Department of Pharmacology, Hospital Group Paris-Saclay, Assistance Publique-Hôpitaux de Paris, France. It was published online on August 30, 2024, in JAMA Network Open.
LIMITATIONS:
The observational nature of the study may have introduced confounding. Current hydroxychloroquine users were younger than nonusers, with an average age difference of almost 5 years. Current hydroxychloroquine users had a twofold longer duration of onset of SLE and had a higher prevalence of chronic kidney disease compared with nonusers.
DISCLOSURES:
This study was funded by the Banque pour l’Investissement, Deeptech. Some authors declared having financial ties with various institutions and companies outside of the current study.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
Managing Vitiligo: Combination Therapies, New Treatments
HUNTINGTON BEACH, CALIFORNIA — When patients with vitiligo see Jessica Shiu, MD, PhD, for the first time, some mention that prior healthcare providers have told them that vitiligo is merely a cosmetic issue — much to her dismay.
“Vitiligo is not a cosmetic disease,” Dr. Shiu, assistant professor of dermatology at the University of California, Irvine, said at the annual meeting of the Pacific Dermatologic Association. “It is associated with significant depression, stigmatization, and low self-esteem. I have patients who say that vitiligo has affected their marriage ... In certain cultures, it also affects their job prospects.”
As the most common pigmentary disorder, vitiligo is an autoimmune condition that often results in the recruitment of CD8+ T cells into the skin. These cells destroy melanocytes, depleting melanocytes in the epidermis. “Over time, this results in milky white patches of skin that we often see in our patients,” Dr. Shiu said.
“Depending on the site that is involved, the nonsegmental form can be further divided into focal, acrofacial, mucosal, generalized, and universal subtypes,” she said. The first step in your initial management is to determine if the vitiligo is active or stable, which can be challenging. Clinical signs of active disease include the presence of trichome vitiligo, confetti vitiligo, and koebnerization.
“Another sign of active disease is when patients tell you that their vitiligo is expanding rapidly,” Dr. Shiu added. “Stable vitiligo is more difficult to define. Many patients think their lesions don’t change, but we’re now appreciating that there can be some sites in those patients such as the hands and feet that are more susceptible to change in activity.” In general, she noted, vitiligo is considered stable when there is no change in activity for at least 12 months, and “lesions are usually completely depigmented with sharp borders.”
The level of vitiligo disease activity drives medical management. For patients with nonsegmental vitiligo who have clinical signs of active disease, the first goal is to stabilize the active disease and stop further spread of depigmentation. “This is key because losing pigment can occur very quickly, but gaining pigment back is a very slow process,” she said. Stabilization involves suppressing immune responses with topical steroids, topical calcineurin inhibitors, or 1.5% ruxolitinib cream, a JAK inhibitor that became the first Food and Drug Administration (FDA)–approved pharmacologic treatment for nonsegmental vitiligo, in 2022, for patients aged 12 years or older.
“The choice here depends somewhat on insurance coverage and shared decision-making with the patient,” Dr. Shiu said. Meanwhile, clinical trials evaluating the effect of the oral JAK inhibitors ritlecitinib, upadacitinib, povorcitinib, and baricitinib on vitiligo are underway.
Combining Phototherapy With Topical Treatment
A mainstay therapy for nonsegmental vitiligo is phototherapy, which can induce the migration of melanocyte stem cells from hair follicles. “There’s good data to show that combining topical treatment with phototherapy can augment the repigmentation that you see,” she said. “So if it’s possible, try to add phototherapy for your vitiligo patients, but sometimes, logistics for that are a challenge.”
Discussing treatment expectations with patients is key because it can take up to 1 year to see a significant response with topical immunosuppressants and narrowband ultraviolet B treatment. The head and neck areas are often the first sites to repigment, she said, followed by the extremities or the trunk. “The hands and feet are generally last; they are usually the most stubborn areas,” Dr. Shiu said. “Even when you do see repigmentation, it usually happens on the dorsal surfaces. The tips of the fingers and toes are difficult to repigment. Luckily, the face is one of the top responders, so that helps a lot.”
While some treatment efforts result in “complete and beautiful” repigmentation, she added, many yield uneven and incomplete results. “We don’t understand why repigmentation occurs in some areas but not in others,” she said. “We don’t have any biomarkers for treatment response. That is something we are looking into.”
For a patient with rapidly progressing active disease, consider an oral steroid mini-pulse 2 consecutive days per week for a maximum of 3-6 months. “I usually recommend that patients do this on Saturday and Sunday,” Dr. Shiu said. “Studies have shown this strategy can halt progression in 85%-91% of cases if patients are on it for at least 3 months.”
Relapse after successful repigmentation occurs in about 40% of cases following discontinuation of treatment, so she recommends biweekly application of 0.1% tacrolimus ointment as maintenance therapy. “Studies have shown this is enough to decrease the relapse rate to around 9%,” she said.
Tissue, Cellular Grafts
Surgical repigmentation strategies rely on transplanting normal skin to areas affected by vitiligo. In general, more than 50% of patients achieve more than 80% repigmentation. Options are divided into tissue grafts vs cellular grafts. “The old methods are tissue grafting such as punch grafting, tissue blister grafting, and spit thickness grafting, which can treat limited areas of skin,” Dr. Shiu said. Newer approaches include cellular grafting using the melanocyte-keratinocyte transplantation procedure, which can treat larger areas of skin.
The main drawback of this approach is that it is expensive and there is no insurance code for it, “but I hope that this becomes an option for our patients in the future because data indicate that repigmentation is maintained for up to 72 months after treatment,” she said.
In June 2023, an autologous cell harvesting device known as RECELL received FDA approval for repigmentation of stable vitiligo lesions. According to a press release from the manufacturer, AVITA Medical, a clinician “prepares and delivers autologous skin cells from pigmented skin to stable depigmented areas, offering a safe and effective treatment for vitiligo.”
Dr. Shiu disclosed that she received research support from AbbVie.
A version of this article first appeared on Medscape.com.
HUNTINGTON BEACH, CALIFORNIA — When patients with vitiligo see Jessica Shiu, MD, PhD, for the first time, some mention that prior healthcare providers have told them that vitiligo is merely a cosmetic issue — much to her dismay.
“Vitiligo is not a cosmetic disease,” Dr. Shiu, assistant professor of dermatology at the University of California, Irvine, said at the annual meeting of the Pacific Dermatologic Association. “It is associated with significant depression, stigmatization, and low self-esteem. I have patients who say that vitiligo has affected their marriage ... In certain cultures, it also affects their job prospects.”
As the most common pigmentary disorder, vitiligo is an autoimmune condition that often results in the recruitment of CD8+ T cells into the skin. These cells destroy melanocytes, depleting melanocytes in the epidermis. “Over time, this results in milky white patches of skin that we often see in our patients,” Dr. Shiu said.
“Depending on the site that is involved, the nonsegmental form can be further divided into focal, acrofacial, mucosal, generalized, and universal subtypes,” she said. The first step in your initial management is to determine if the vitiligo is active or stable, which can be challenging. Clinical signs of active disease include the presence of trichome vitiligo, confetti vitiligo, and koebnerization.
“Another sign of active disease is when patients tell you that their vitiligo is expanding rapidly,” Dr. Shiu added. “Stable vitiligo is more difficult to define. Many patients think their lesions don’t change, but we’re now appreciating that there can be some sites in those patients such as the hands and feet that are more susceptible to change in activity.” In general, she noted, vitiligo is considered stable when there is no change in activity for at least 12 months, and “lesions are usually completely depigmented with sharp borders.”
The level of vitiligo disease activity drives medical management. For patients with nonsegmental vitiligo who have clinical signs of active disease, the first goal is to stabilize the active disease and stop further spread of depigmentation. “This is key because losing pigment can occur very quickly, but gaining pigment back is a very slow process,” she said. Stabilization involves suppressing immune responses with topical steroids, topical calcineurin inhibitors, or 1.5% ruxolitinib cream, a JAK inhibitor that became the first Food and Drug Administration (FDA)–approved pharmacologic treatment for nonsegmental vitiligo, in 2022, for patients aged 12 years or older.
“The choice here depends somewhat on insurance coverage and shared decision-making with the patient,” Dr. Shiu said. Meanwhile, clinical trials evaluating the effect of the oral JAK inhibitors ritlecitinib, upadacitinib, povorcitinib, and baricitinib on vitiligo are underway.
Combining Phototherapy With Topical Treatment
A mainstay therapy for nonsegmental vitiligo is phototherapy, which can induce the migration of melanocyte stem cells from hair follicles. “There’s good data to show that combining topical treatment with phototherapy can augment the repigmentation that you see,” she said. “So if it’s possible, try to add phototherapy for your vitiligo patients, but sometimes, logistics for that are a challenge.”
Discussing treatment expectations with patients is key because it can take up to 1 year to see a significant response with topical immunosuppressants and narrowband ultraviolet B treatment. The head and neck areas are often the first sites to repigment, she said, followed by the extremities or the trunk. “The hands and feet are generally last; they are usually the most stubborn areas,” Dr. Shiu said. “Even when you do see repigmentation, it usually happens on the dorsal surfaces. The tips of the fingers and toes are difficult to repigment. Luckily, the face is one of the top responders, so that helps a lot.”
While some treatment efforts result in “complete and beautiful” repigmentation, she added, many yield uneven and incomplete results. “We don’t understand why repigmentation occurs in some areas but not in others,” she said. “We don’t have any biomarkers for treatment response. That is something we are looking into.”
For a patient with rapidly progressing active disease, consider an oral steroid mini-pulse 2 consecutive days per week for a maximum of 3-6 months. “I usually recommend that patients do this on Saturday and Sunday,” Dr. Shiu said. “Studies have shown this strategy can halt progression in 85%-91% of cases if patients are on it for at least 3 months.”
Relapse after successful repigmentation occurs in about 40% of cases following discontinuation of treatment, so she recommends biweekly application of 0.1% tacrolimus ointment as maintenance therapy. “Studies have shown this is enough to decrease the relapse rate to around 9%,” she said.
Tissue, Cellular Grafts
Surgical repigmentation strategies rely on transplanting normal skin to areas affected by vitiligo. In general, more than 50% of patients achieve more than 80% repigmentation. Options are divided into tissue grafts vs cellular grafts. “The old methods are tissue grafting such as punch grafting, tissue blister grafting, and spit thickness grafting, which can treat limited areas of skin,” Dr. Shiu said. Newer approaches include cellular grafting using the melanocyte-keratinocyte transplantation procedure, which can treat larger areas of skin.
The main drawback of this approach is that it is expensive and there is no insurance code for it, “but I hope that this becomes an option for our patients in the future because data indicate that repigmentation is maintained for up to 72 months after treatment,” she said.
In June 2023, an autologous cell harvesting device known as RECELL received FDA approval for repigmentation of stable vitiligo lesions. According to a press release from the manufacturer, AVITA Medical, a clinician “prepares and delivers autologous skin cells from pigmented skin to stable depigmented areas, offering a safe and effective treatment for vitiligo.”
Dr. Shiu disclosed that she received research support from AbbVie.
A version of this article first appeared on Medscape.com.
HUNTINGTON BEACH, CALIFORNIA — When patients with vitiligo see Jessica Shiu, MD, PhD, for the first time, some mention that prior healthcare providers have told them that vitiligo is merely a cosmetic issue — much to her dismay.
“Vitiligo is not a cosmetic disease,” Dr. Shiu, assistant professor of dermatology at the University of California, Irvine, said at the annual meeting of the Pacific Dermatologic Association. “It is associated with significant depression, stigmatization, and low self-esteem. I have patients who say that vitiligo has affected their marriage ... In certain cultures, it also affects their job prospects.”
As the most common pigmentary disorder, vitiligo is an autoimmune condition that often results in the recruitment of CD8+ T cells into the skin. These cells destroy melanocytes, depleting melanocytes in the epidermis. “Over time, this results in milky white patches of skin that we often see in our patients,” Dr. Shiu said.
“Depending on the site that is involved, the nonsegmental form can be further divided into focal, acrofacial, mucosal, generalized, and universal subtypes,” she said. The first step in your initial management is to determine if the vitiligo is active or stable, which can be challenging. Clinical signs of active disease include the presence of trichome vitiligo, confetti vitiligo, and koebnerization.
“Another sign of active disease is when patients tell you that their vitiligo is expanding rapidly,” Dr. Shiu added. “Stable vitiligo is more difficult to define. Many patients think their lesions don’t change, but we’re now appreciating that there can be some sites in those patients such as the hands and feet that are more susceptible to change in activity.” In general, she noted, vitiligo is considered stable when there is no change in activity for at least 12 months, and “lesions are usually completely depigmented with sharp borders.”
The level of vitiligo disease activity drives medical management. For patients with nonsegmental vitiligo who have clinical signs of active disease, the first goal is to stabilize the active disease and stop further spread of depigmentation. “This is key because losing pigment can occur very quickly, but gaining pigment back is a very slow process,” she said. Stabilization involves suppressing immune responses with topical steroids, topical calcineurin inhibitors, or 1.5% ruxolitinib cream, a JAK inhibitor that became the first Food and Drug Administration (FDA)–approved pharmacologic treatment for nonsegmental vitiligo, in 2022, for patients aged 12 years or older.
“The choice here depends somewhat on insurance coverage and shared decision-making with the patient,” Dr. Shiu said. Meanwhile, clinical trials evaluating the effect of the oral JAK inhibitors ritlecitinib, upadacitinib, povorcitinib, and baricitinib on vitiligo are underway.
Combining Phototherapy With Topical Treatment
A mainstay therapy for nonsegmental vitiligo is phototherapy, which can induce the migration of melanocyte stem cells from hair follicles. “There’s good data to show that combining topical treatment with phototherapy can augment the repigmentation that you see,” she said. “So if it’s possible, try to add phototherapy for your vitiligo patients, but sometimes, logistics for that are a challenge.”
Discussing treatment expectations with patients is key because it can take up to 1 year to see a significant response with topical immunosuppressants and narrowband ultraviolet B treatment. The head and neck areas are often the first sites to repigment, she said, followed by the extremities or the trunk. “The hands and feet are generally last; they are usually the most stubborn areas,” Dr. Shiu said. “Even when you do see repigmentation, it usually happens on the dorsal surfaces. The tips of the fingers and toes are difficult to repigment. Luckily, the face is one of the top responders, so that helps a lot.”
While some treatment efforts result in “complete and beautiful” repigmentation, she added, many yield uneven and incomplete results. “We don’t understand why repigmentation occurs in some areas but not in others,” she said. “We don’t have any biomarkers for treatment response. That is something we are looking into.”
For a patient with rapidly progressing active disease, consider an oral steroid mini-pulse 2 consecutive days per week for a maximum of 3-6 months. “I usually recommend that patients do this on Saturday and Sunday,” Dr. Shiu said. “Studies have shown this strategy can halt progression in 85%-91% of cases if patients are on it for at least 3 months.”
Relapse after successful repigmentation occurs in about 40% of cases following discontinuation of treatment, so she recommends biweekly application of 0.1% tacrolimus ointment as maintenance therapy. “Studies have shown this is enough to decrease the relapse rate to around 9%,” she said.
Tissue, Cellular Grafts
Surgical repigmentation strategies rely on transplanting normal skin to areas affected by vitiligo. In general, more than 50% of patients achieve more than 80% repigmentation. Options are divided into tissue grafts vs cellular grafts. “The old methods are tissue grafting such as punch grafting, tissue blister grafting, and spit thickness grafting, which can treat limited areas of skin,” Dr. Shiu said. Newer approaches include cellular grafting using the melanocyte-keratinocyte transplantation procedure, which can treat larger areas of skin.
The main drawback of this approach is that it is expensive and there is no insurance code for it, “but I hope that this becomes an option for our patients in the future because data indicate that repigmentation is maintained for up to 72 months after treatment,” she said.
In June 2023, an autologous cell harvesting device known as RECELL received FDA approval for repigmentation of stable vitiligo lesions. According to a press release from the manufacturer, AVITA Medical, a clinician “prepares and delivers autologous skin cells from pigmented skin to stable depigmented areas, offering a safe and effective treatment for vitiligo.”
Dr. Shiu disclosed that she received research support from AbbVie.
A version of this article first appeared on Medscape.com.
FROM PDA 2024
What’s Causing Raynaud Phenomenon Severity to Rise With High Temperatures?
TOPLINE:
In systemic sclerosis, Raynaud phenomenon is more severe at both high and low temperature extremes, according to new research.
BACKGROUND:
- Raynaud phenomenon, a condition that causes decreased blood flow to extremities, occurs in about 95% of individuals with systemic sclerosis.
- Episodes of Raynaud phenomenon can be triggered by cold exposure and ambient temperature changes.
- In severe cases, it can cause permanent damage to tissues of the fingers and toes.
METHODOLOGY:
- Researchers analyzed data from 2243 participants with Raynaud phenomenon secondary to systemic sclerosis from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort.
- Participants completed past-week Raynaud phenomenon severity assessments using a 0-10 numerical rating scale at enrollment and every 3 months.
- The study included data from 20,233 Raynaud phenomenon severity assessments between April 15, 2014, and August 1, 2023.
- Researchers used average daily temperature from a weather site close to the participant’s recruiting center and mapped these ambient temperature changes to Raynaud’s phenomenon outcomes.
TAKEAWAY:
- Raynaud’s phenomenon severity was highest at –25 °C (–13 °F), with assessment scores at 6.8 points out of 10.0, and lowest at 25 °C (77 °F), with scores at 2.6.
- Severity scores increased again at temperatures above 35 °C (95 °F), reaching a high of 5.6 out of 10 at 40 °C (104 °F).
- This spike at higher temperatures is presumably due to air conditioning, the authors said.
- In an accompanying commentary, Cutolo et al. posited that increased sweating and hypotension could also lead to a relative hypovolemic state in patients, causing Raynaud-like symptoms.
IN PRACTICE:
“Temperature-related variations in Raynaud’s phenomenon severity scores should be considered in clinical trials to account for normal within-season temperature fluctuations, enhancing the accuracy of treatment outcomes,” wrote Cutolo and colleagues in their commentary.
SOURCE:
The study was led by Gabrielle Virgili-Gervais, MSc, McGill University Health Centre in Montreal, Quebec, Canada. It was published online on August 28 in The Lancet Rheumatology. The accompanying commentary, also published on August 28, was authored by Maurizio Cutolo, MD, and Elvis Hysa, MD, both of University of Genova, Italy, as well as Vanessa Smith, MD, PhD, of Ghent University in Ghent, Belgium.
LIMITATIONS:
The lower number of assessments at extreme temperatures (–25 °C and 40 °C) may affect the robustness of the findings at these ranges. The study did not account for vasodilator use, which could influence participants’ response to temperature. The study also did not account for other potential confounding factors such as sex, smoking status, psychosocial factors, and comorbid conditions like cardiovascular disease.
DISCLOSURES:
A variety of scleroderma-related patient advocacy groups helped to fund research on the SPIN cohort, in addition to the Canadian Institutes of Health Research, the Arthritis Society, the Lady Davis Institute for Medical Research of the Jewish General Hospital, the Jewish General Hospital Foundation, and McGill University. Two authors reported having financial ties with pharmaceutical companies. Dr. Cutolo, Dr. Smith, and Dr. Hysa had no disclosures.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
TOPLINE:
In systemic sclerosis, Raynaud phenomenon is more severe at both high and low temperature extremes, according to new research.
BACKGROUND:
- Raynaud phenomenon, a condition that causes decreased blood flow to extremities, occurs in about 95% of individuals with systemic sclerosis.
- Episodes of Raynaud phenomenon can be triggered by cold exposure and ambient temperature changes.
- In severe cases, it can cause permanent damage to tissues of the fingers and toes.
METHODOLOGY:
- Researchers analyzed data from 2243 participants with Raynaud phenomenon secondary to systemic sclerosis from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort.
- Participants completed past-week Raynaud phenomenon severity assessments using a 0-10 numerical rating scale at enrollment and every 3 months.
- The study included data from 20,233 Raynaud phenomenon severity assessments between April 15, 2014, and August 1, 2023.
- Researchers used average daily temperature from a weather site close to the participant’s recruiting center and mapped these ambient temperature changes to Raynaud’s phenomenon outcomes.
TAKEAWAY:
- Raynaud’s phenomenon severity was highest at –25 °C (–13 °F), with assessment scores at 6.8 points out of 10.0, and lowest at 25 °C (77 °F), with scores at 2.6.
- Severity scores increased again at temperatures above 35 °C (95 °F), reaching a high of 5.6 out of 10 at 40 °C (104 °F).
- This spike at higher temperatures is presumably due to air conditioning, the authors said.
- In an accompanying commentary, Cutolo et al. posited that increased sweating and hypotension could also lead to a relative hypovolemic state in patients, causing Raynaud-like symptoms.
IN PRACTICE:
“Temperature-related variations in Raynaud’s phenomenon severity scores should be considered in clinical trials to account for normal within-season temperature fluctuations, enhancing the accuracy of treatment outcomes,” wrote Cutolo and colleagues in their commentary.
SOURCE:
The study was led by Gabrielle Virgili-Gervais, MSc, McGill University Health Centre in Montreal, Quebec, Canada. It was published online on August 28 in The Lancet Rheumatology. The accompanying commentary, also published on August 28, was authored by Maurizio Cutolo, MD, and Elvis Hysa, MD, both of University of Genova, Italy, as well as Vanessa Smith, MD, PhD, of Ghent University in Ghent, Belgium.
LIMITATIONS:
The lower number of assessments at extreme temperatures (–25 °C and 40 °C) may affect the robustness of the findings at these ranges. The study did not account for vasodilator use, which could influence participants’ response to temperature. The study also did not account for other potential confounding factors such as sex, smoking status, psychosocial factors, and comorbid conditions like cardiovascular disease.
DISCLOSURES:
A variety of scleroderma-related patient advocacy groups helped to fund research on the SPIN cohort, in addition to the Canadian Institutes of Health Research, the Arthritis Society, the Lady Davis Institute for Medical Research of the Jewish General Hospital, the Jewish General Hospital Foundation, and McGill University. Two authors reported having financial ties with pharmaceutical companies. Dr. Cutolo, Dr. Smith, and Dr. Hysa had no disclosures.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
TOPLINE:
In systemic sclerosis, Raynaud phenomenon is more severe at both high and low temperature extremes, according to new research.
BACKGROUND:
- Raynaud phenomenon, a condition that causes decreased blood flow to extremities, occurs in about 95% of individuals with systemic sclerosis.
- Episodes of Raynaud phenomenon can be triggered by cold exposure and ambient temperature changes.
- In severe cases, it can cause permanent damage to tissues of the fingers and toes.
METHODOLOGY:
- Researchers analyzed data from 2243 participants with Raynaud phenomenon secondary to systemic sclerosis from the Scleroderma Patient-centered Intervention Network (SPIN) Cohort.
- Participants completed past-week Raynaud phenomenon severity assessments using a 0-10 numerical rating scale at enrollment and every 3 months.
- The study included data from 20,233 Raynaud phenomenon severity assessments between April 15, 2014, and August 1, 2023.
- Researchers used average daily temperature from a weather site close to the participant’s recruiting center and mapped these ambient temperature changes to Raynaud’s phenomenon outcomes.
TAKEAWAY:
- Raynaud’s phenomenon severity was highest at –25 °C (–13 °F), with assessment scores at 6.8 points out of 10.0, and lowest at 25 °C (77 °F), with scores at 2.6.
- Severity scores increased again at temperatures above 35 °C (95 °F), reaching a high of 5.6 out of 10 at 40 °C (104 °F).
- This spike at higher temperatures is presumably due to air conditioning, the authors said.
- In an accompanying commentary, Cutolo et al. posited that increased sweating and hypotension could also lead to a relative hypovolemic state in patients, causing Raynaud-like symptoms.
IN PRACTICE:
“Temperature-related variations in Raynaud’s phenomenon severity scores should be considered in clinical trials to account for normal within-season temperature fluctuations, enhancing the accuracy of treatment outcomes,” wrote Cutolo and colleagues in their commentary.
SOURCE:
The study was led by Gabrielle Virgili-Gervais, MSc, McGill University Health Centre in Montreal, Quebec, Canada. It was published online on August 28 in The Lancet Rheumatology. The accompanying commentary, also published on August 28, was authored by Maurizio Cutolo, MD, and Elvis Hysa, MD, both of University of Genova, Italy, as well as Vanessa Smith, MD, PhD, of Ghent University in Ghent, Belgium.
LIMITATIONS:
The lower number of assessments at extreme temperatures (–25 °C and 40 °C) may affect the robustness of the findings at these ranges. The study did not account for vasodilator use, which could influence participants’ response to temperature. The study also did not account for other potential confounding factors such as sex, smoking status, psychosocial factors, and comorbid conditions like cardiovascular disease.
DISCLOSURES:
A variety of scleroderma-related patient advocacy groups helped to fund research on the SPIN cohort, in addition to the Canadian Institutes of Health Research, the Arthritis Society, the Lady Davis Institute for Medical Research of the Jewish General Hospital, the Jewish General Hospital Foundation, and McGill University. Two authors reported having financial ties with pharmaceutical companies. Dr. Cutolo, Dr. Smith, and Dr. Hysa had no disclosures.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
Systemic Sclerosis Without Scleroderma Has Unique Severity, Prognosis
TOPLINE:
Systemic sclerosis sine scleroderma (ssSSc) affects nearly 10% of patients with systemic sclerosis (SSc), with substantial internal organ involvement. Despite lacking skin fibrosis, patients with ssSSc are at a risk for interstitial lung disease, pulmonary arterial hypertension, and cardiac dysfunction.
METHODOLOGY:
- Driven by a fatal case of ssSSc with cardiac involvement, researchers aimed to evaluate its prevalence, severity, and prognosis.
- They conducted a systematic literature and qualitative synthesis of 35 studies on SSc cohorts from databases published between 1976 and 2023 that comprised data on the prevalence of SSc with or without organ involvement.
- A total of 25,455 patients with SSc were included, with 2437 identified as having ssSSc.
- Studies used various classification criteria for SSc, including the 1980 American Rheumatism Association criteria, 2001 LeRoy and Medsger criteria, and 2013 American College of Rheumatology/European League Against Rheumatism criteria, while ssSSc was classified on the basis of the definitions provided by Rodnan and Fennell and also Poormoghim.
- The analysis focused on ssSSc prevalence, reclassification rates, and internal organ involvement, including interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and cardiac dysfunction.
TAKEAWAY:
- The overall mean prevalence of ssSSc was 9.6%, with a range of 0%-22.9% across different studies.
- Reclassification rates of ssSSc into limited cutaneous SSc (lcSSc) or diffuse cutaneous SSc (dcSSc) varied substantially, with some studies reporting rates as high as 27.8% over a 4-year follow-up period.
- The mean frequency of internal organ involvement in patients with ssSSc was 46% for interstitial lung disease, 15% for pulmonary arterial hypertension, 5% for scleroderma renal crisis, and 26.5% for cardiac dysfunction — mainly diastolic dysfunction.
- The survival rates in patients with ssSSc were similar to those with lcSSc and better than those with dcSSc.
IN PRACTICE:
“The results presented herein suggest a slightly more severe yet similar clinical picture of ssSSc compared to lcSSc [limited cutaneous SSc], while dcSSc [diffuse cutaneous SSc] remains the most severe disease form,” the authors wrote. “Although classification criteria should not impact appropriate management of patients, updated ssSSc subclassification criteria, which will take into account time from disease onset, should be considered,” they further added.
SOURCE:
The study was led by Anastasios Makris, MD, First Department of Propaedeutic & Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece. It was published online on August 15, 2024, in The Journal of Rheumatology.
LIMITATIONS:
The variability in the classification criteria across different studies may affect the comparability of results. The included studies lacked data on cardiac MRI, restricting the identification of myocardial fibrosis patterns and characterization of cardiac disease activity.
DISCLOSURES:
The study did not receive any specific funding. Some authors disclosed having a consultancy relationship, serving as speakers, and receiving funding for research from multiple companies. One author reported having a patent and being a cofounder of CITUS AG.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Systemic sclerosis sine scleroderma (ssSSc) affects nearly 10% of patients with systemic sclerosis (SSc), with substantial internal organ involvement. Despite lacking skin fibrosis, patients with ssSSc are at a risk for interstitial lung disease, pulmonary arterial hypertension, and cardiac dysfunction.
METHODOLOGY:
- Driven by a fatal case of ssSSc with cardiac involvement, researchers aimed to evaluate its prevalence, severity, and prognosis.
- They conducted a systematic literature and qualitative synthesis of 35 studies on SSc cohorts from databases published between 1976 and 2023 that comprised data on the prevalence of SSc with or without organ involvement.
- A total of 25,455 patients with SSc were included, with 2437 identified as having ssSSc.
- Studies used various classification criteria for SSc, including the 1980 American Rheumatism Association criteria, 2001 LeRoy and Medsger criteria, and 2013 American College of Rheumatology/European League Against Rheumatism criteria, while ssSSc was classified on the basis of the definitions provided by Rodnan and Fennell and also Poormoghim.
- The analysis focused on ssSSc prevalence, reclassification rates, and internal organ involvement, including interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and cardiac dysfunction.
TAKEAWAY:
- The overall mean prevalence of ssSSc was 9.6%, with a range of 0%-22.9% across different studies.
- Reclassification rates of ssSSc into limited cutaneous SSc (lcSSc) or diffuse cutaneous SSc (dcSSc) varied substantially, with some studies reporting rates as high as 27.8% over a 4-year follow-up period.
- The mean frequency of internal organ involvement in patients with ssSSc was 46% for interstitial lung disease, 15% for pulmonary arterial hypertension, 5% for scleroderma renal crisis, and 26.5% for cardiac dysfunction — mainly diastolic dysfunction.
- The survival rates in patients with ssSSc were similar to those with lcSSc and better than those with dcSSc.
IN PRACTICE:
“The results presented herein suggest a slightly more severe yet similar clinical picture of ssSSc compared to lcSSc [limited cutaneous SSc], while dcSSc [diffuse cutaneous SSc] remains the most severe disease form,” the authors wrote. “Although classification criteria should not impact appropriate management of patients, updated ssSSc subclassification criteria, which will take into account time from disease onset, should be considered,” they further added.
SOURCE:
The study was led by Anastasios Makris, MD, First Department of Propaedeutic & Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece. It was published online on August 15, 2024, in The Journal of Rheumatology.
LIMITATIONS:
The variability in the classification criteria across different studies may affect the comparability of results. The included studies lacked data on cardiac MRI, restricting the identification of myocardial fibrosis patterns and characterization of cardiac disease activity.
DISCLOSURES:
The study did not receive any specific funding. Some authors disclosed having a consultancy relationship, serving as speakers, and receiving funding for research from multiple companies. One author reported having a patent and being a cofounder of CITUS AG.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
TOPLINE:
Systemic sclerosis sine scleroderma (ssSSc) affects nearly 10% of patients with systemic sclerosis (SSc), with substantial internal organ involvement. Despite lacking skin fibrosis, patients with ssSSc are at a risk for interstitial lung disease, pulmonary arterial hypertension, and cardiac dysfunction.
METHODOLOGY:
- Driven by a fatal case of ssSSc with cardiac involvement, researchers aimed to evaluate its prevalence, severity, and prognosis.
- They conducted a systematic literature and qualitative synthesis of 35 studies on SSc cohorts from databases published between 1976 and 2023 that comprised data on the prevalence of SSc with or without organ involvement.
- A total of 25,455 patients with SSc were included, with 2437 identified as having ssSSc.
- Studies used various classification criteria for SSc, including the 1980 American Rheumatism Association criteria, 2001 LeRoy and Medsger criteria, and 2013 American College of Rheumatology/European League Against Rheumatism criteria, while ssSSc was classified on the basis of the definitions provided by Rodnan and Fennell and also Poormoghim.
- The analysis focused on ssSSc prevalence, reclassification rates, and internal organ involvement, including interstitial lung disease, pulmonary arterial hypertension, scleroderma renal crisis, and cardiac dysfunction.
TAKEAWAY:
- The overall mean prevalence of ssSSc was 9.6%, with a range of 0%-22.9% across different studies.
- Reclassification rates of ssSSc into limited cutaneous SSc (lcSSc) or diffuse cutaneous SSc (dcSSc) varied substantially, with some studies reporting rates as high as 27.8% over a 4-year follow-up period.
- The mean frequency of internal organ involvement in patients with ssSSc was 46% for interstitial lung disease, 15% for pulmonary arterial hypertension, 5% for scleroderma renal crisis, and 26.5% for cardiac dysfunction — mainly diastolic dysfunction.
- The survival rates in patients with ssSSc were similar to those with lcSSc and better than those with dcSSc.
IN PRACTICE:
“The results presented herein suggest a slightly more severe yet similar clinical picture of ssSSc compared to lcSSc [limited cutaneous SSc], while dcSSc [diffuse cutaneous SSc] remains the most severe disease form,” the authors wrote. “Although classification criteria should not impact appropriate management of patients, updated ssSSc subclassification criteria, which will take into account time from disease onset, should be considered,” they further added.
SOURCE:
The study was led by Anastasios Makris, MD, First Department of Propaedeutic & Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece. It was published online on August 15, 2024, in The Journal of Rheumatology.
LIMITATIONS:
The variability in the classification criteria across different studies may affect the comparability of results. The included studies lacked data on cardiac MRI, restricting the identification of myocardial fibrosis patterns and characterization of cardiac disease activity.
DISCLOSURES:
The study did not receive any specific funding. Some authors disclosed having a consultancy relationship, serving as speakers, and receiving funding for research from multiple companies. One author reported having a patent and being a cofounder of CITUS AG.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article appeared on Medscape.com.
Patients With Immune-Mediated Inflammatory Diseases, Type 2 Diabetes Reap GLP-1 Receptor Agonist Benefits, Too
TOPLINE:
Compared with dipeptidyl peptidase 4 (DPP-4) inhibitors, glucagon-like peptide 1 receptor agonists (GLP-1 RAs) are associated with a lower risk for all-cause mortality and major adverse cardiovascular events (MACE) in patients with immune-mediated inflammatory diseases (IMIDs) and type 2 diabetes (T2D).
METHODOLOGY:
- GLP-1 RAs reduce the risk for all-cause mortality, cardiovascular mortality, and stroke in patients with diabetes. However, previous trials have excluded those with IMIDs, leaving a gap in understanding the cardioprotective effects of GLP-1 RAs in this population.
- Researchers conducted a population-based cohort study to assess if patients with an IMID derive greater benefits from GLP-1 RAs than DPP-4 inhibitors.
- They used administrative health data from British Columbia, Canada, to include 10,855 patients with IMIDs (rheumatoid arthritis, psoriatic disease, ankylosing spondylitis, inflammatory bowel disease, or systemic autoimmune rheumatic disease) and T2D who initiated either GLP-1 RA (n = 3570) or DPP-4 inhibitor (n = 7285).
- The mean follow-up was 1.46 and 1.88 years in the GLP-1 RA and DPP-4 inhibitor cohorts, respectively.
- The primary outcome was all-cause mortality, and the secondary outcome was MACE, including cardiovascular death, myocardial infarction, and ischemic stroke.
TAKEAWAY:
- The risk for all-cause mortality was 52% lower in patients who initiated GLP-1 RAs than in those who initiated DPP-4 inhibitors (weighted hazard ratio [HR], 0.48; 95% CI, 0.31-0.75).
- Additionally, patients initiating DPP-4 inhibitors.
- In the subgroup of patients with GLP-1 RAs had a significantly lower risk for MACE (weighted HR, 0.66; 95% CI, 0.50-0.88), particularly myocardial infarction (weighted HR, 0.62; 95% CI, 0.40-0.96), than those initiating rheumatoid arthritis and T2D, those who initiated GLP-1 RAs had a 55% lower risk for all-cause mortality and 61% lower risk for MACE than those who initiated DPP-4 inhibitors.
IN PRACTICE:
“This corresponds to nine fewer deaths and 11 fewer MACE per 1000 person-years, respectively, supporting the hypothesis that these agents have a cardioprotective effect in this high-risk population,” the authors wrote.
SOURCE:
This study was led by Derin Karacabeyli, MD, Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada, and was published online on August 8, 2024, in PLOS ONE.
LIMITATIONS:
The study’s dependence on administrative health data might have resulted in incomplete capture of comorbidities, particularly obesity. The mean follow-up period was relatively short, which might have limited the long-term applicability of these findings. The accuracy of the case definitions for IMIDs and T2D, according to International Classification of Diseases codes, could not be fully ascertained.
DISCLOSURES:
The study was supported by grants from the Canadian Institutes of Health Research. Two authors declared receiving research support, consulting fees, or participating in advisory boards outside the submitted work.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
TOPLINE:
Compared with dipeptidyl peptidase 4 (DPP-4) inhibitors, glucagon-like peptide 1 receptor agonists (GLP-1 RAs) are associated with a lower risk for all-cause mortality and major adverse cardiovascular events (MACE) in patients with immune-mediated inflammatory diseases (IMIDs) and type 2 diabetes (T2D).
METHODOLOGY:
- GLP-1 RAs reduce the risk for all-cause mortality, cardiovascular mortality, and stroke in patients with diabetes. However, previous trials have excluded those with IMIDs, leaving a gap in understanding the cardioprotective effects of GLP-1 RAs in this population.
- Researchers conducted a population-based cohort study to assess if patients with an IMID derive greater benefits from GLP-1 RAs than DPP-4 inhibitors.
- They used administrative health data from British Columbia, Canada, to include 10,855 patients with IMIDs (rheumatoid arthritis, psoriatic disease, ankylosing spondylitis, inflammatory bowel disease, or systemic autoimmune rheumatic disease) and T2D who initiated either GLP-1 RA (n = 3570) or DPP-4 inhibitor (n = 7285).
- The mean follow-up was 1.46 and 1.88 years in the GLP-1 RA and DPP-4 inhibitor cohorts, respectively.
- The primary outcome was all-cause mortality, and the secondary outcome was MACE, including cardiovascular death, myocardial infarction, and ischemic stroke.
TAKEAWAY:
- The risk for all-cause mortality was 52% lower in patients who initiated GLP-1 RAs than in those who initiated DPP-4 inhibitors (weighted hazard ratio [HR], 0.48; 95% CI, 0.31-0.75).
- Additionally, patients initiating DPP-4 inhibitors.
- In the subgroup of patients with GLP-1 RAs had a significantly lower risk for MACE (weighted HR, 0.66; 95% CI, 0.50-0.88), particularly myocardial infarction (weighted HR, 0.62; 95% CI, 0.40-0.96), than those initiating rheumatoid arthritis and T2D, those who initiated GLP-1 RAs had a 55% lower risk for all-cause mortality and 61% lower risk for MACE than those who initiated DPP-4 inhibitors.
IN PRACTICE:
“This corresponds to nine fewer deaths and 11 fewer MACE per 1000 person-years, respectively, supporting the hypothesis that these agents have a cardioprotective effect in this high-risk population,” the authors wrote.
SOURCE:
This study was led by Derin Karacabeyli, MD, Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada, and was published online on August 8, 2024, in PLOS ONE.
LIMITATIONS:
The study’s dependence on administrative health data might have resulted in incomplete capture of comorbidities, particularly obesity. The mean follow-up period was relatively short, which might have limited the long-term applicability of these findings. The accuracy of the case definitions for IMIDs and T2D, according to International Classification of Diseases codes, could not be fully ascertained.
DISCLOSURES:
The study was supported by grants from the Canadian Institutes of Health Research. Two authors declared receiving research support, consulting fees, or participating in advisory boards outside the submitted work.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
TOPLINE:
Compared with dipeptidyl peptidase 4 (DPP-4) inhibitors, glucagon-like peptide 1 receptor agonists (GLP-1 RAs) are associated with a lower risk for all-cause mortality and major adverse cardiovascular events (MACE) in patients with immune-mediated inflammatory diseases (IMIDs) and type 2 diabetes (T2D).
METHODOLOGY:
- GLP-1 RAs reduce the risk for all-cause mortality, cardiovascular mortality, and stroke in patients with diabetes. However, previous trials have excluded those with IMIDs, leaving a gap in understanding the cardioprotective effects of GLP-1 RAs in this population.
- Researchers conducted a population-based cohort study to assess if patients with an IMID derive greater benefits from GLP-1 RAs than DPP-4 inhibitors.
- They used administrative health data from British Columbia, Canada, to include 10,855 patients with IMIDs (rheumatoid arthritis, psoriatic disease, ankylosing spondylitis, inflammatory bowel disease, or systemic autoimmune rheumatic disease) and T2D who initiated either GLP-1 RA (n = 3570) or DPP-4 inhibitor (n = 7285).
- The mean follow-up was 1.46 and 1.88 years in the GLP-1 RA and DPP-4 inhibitor cohorts, respectively.
- The primary outcome was all-cause mortality, and the secondary outcome was MACE, including cardiovascular death, myocardial infarction, and ischemic stroke.
TAKEAWAY:
- The risk for all-cause mortality was 52% lower in patients who initiated GLP-1 RAs than in those who initiated DPP-4 inhibitors (weighted hazard ratio [HR], 0.48; 95% CI, 0.31-0.75).
- Additionally, patients initiating DPP-4 inhibitors.
- In the subgroup of patients with GLP-1 RAs had a significantly lower risk for MACE (weighted HR, 0.66; 95% CI, 0.50-0.88), particularly myocardial infarction (weighted HR, 0.62; 95% CI, 0.40-0.96), than those initiating rheumatoid arthritis and T2D, those who initiated GLP-1 RAs had a 55% lower risk for all-cause mortality and 61% lower risk for MACE than those who initiated DPP-4 inhibitors.
IN PRACTICE:
“This corresponds to nine fewer deaths and 11 fewer MACE per 1000 person-years, respectively, supporting the hypothesis that these agents have a cardioprotective effect in this high-risk population,” the authors wrote.
SOURCE:
This study was led by Derin Karacabeyli, MD, Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada, and was published online on August 8, 2024, in PLOS ONE.
LIMITATIONS:
The study’s dependence on administrative health data might have resulted in incomplete capture of comorbidities, particularly obesity. The mean follow-up period was relatively short, which might have limited the long-term applicability of these findings. The accuracy of the case definitions for IMIDs and T2D, according to International Classification of Diseases codes, could not be fully ascertained.
DISCLOSURES:
The study was supported by grants from the Canadian Institutes of Health Research. Two authors declared receiving research support, consulting fees, or participating in advisory boards outside the submitted work.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication. A version of this article first appeared on Medscape.com.
Fillers, Hyaluronidase Relieve Orofacial Changes in Patients with Scleroderma
CARLSBAD, CALIFORNIA — In 2003, researchers asked 303 patients with systemic sclerosis (scleroderma) what bothered them most about their disease from an aesthetic standpoint: Orofacial features, such as thin lips and mouth furrows, or non-facial features, such as fingertip ulceration and waxy changes to the skin.
Respondents expressed significant concern about specific orofacial features, including thin lips (73%), mouth furrows (80%), loss of facial lines (68%), and a smaller, tighter mouth (77%).
“Patients with systemic sclerosis may have loss of vermilion lip, microstomia, and perioral rhytids,” Kathleen Cook Suozzi, MD, who directs the Aesthetic Dermatology Program at Yale University School of Medicine, New Haven, Connecticut, said at the Controversies and Conversations in Laser and Cosmetic Surgery annual symposium. “How can we address these changes for our patients?”
Recent research has shown that hyaluronidase injections can help improve orofacial changes commonly experienced by patients with scleroderma. In 2019, researchers in Alabama reported the case of a 53-year-old woman treated with hyaluronidase for scleroderma-induced microstomia. After four visits over 7 months and a total hyaluronidase dose of 470 IU, the patient reported an improved Mouth Handicap in Systemic Sclerosis (MHISS) score (38 of 48); subjective improvement of symptoms, including greater ease in eating and undergoing dental treatment; and improved mouth closure.
In 2023, researchers published a cohort study of four women between the ages of 43 and 61 with autoimmune sclerosing conditions that resulted in oral microstomia. Following hyaluronidase injections, all improved in mouth opening capacity and MHISS, with change stabilizing between three and five treatments. More recently, in a study pending publication in JAAD Case Reports, Dr. Suozzi and colleagues retrospectively evaluated 12 women with scleroderma who received between 150 and 300 units of hyaluronic acid (HA) filler for microstomia between 2020 and 2023. Of the 12 women, 58% had diffuse disease, and 42% had limited disease. Overall, oral aperture width increased by 0.65 cm (P = .0027) and oral aperture height increased by 0.88 cm (P < .0001). “In general, patients needed three to four treatments to reach peak effect, and then they reached a plateau,” Dr. Suozzi said. “It wasn’t that the treatment wasn’t working anymore, but it was because their oral aperture had gotten to a size of around 5 cm, which is clinically normal. Interestingly, we found that if the patient’s disease flared and their microstomia started to return, when you rechallenged them, they continued to respond. So, patients can continue to use this treatment over time.”
In a separate case series of seven patients, Dr. Suozzi and colleagues prospectively evaluated the effect of HA soft tissue filler with Restylane Silk for lip augmentation. Study participants experienced statistically significant increases in the difference between pre- and postinjection fullness in both upper and lower lips. Also, the mean posttreatment score fell between “much improved” (2) and “improved” (3) on both the Investigator Global Aesthetic Improvement Scale and the Subject Global Aesthetic Improvement Scale.
Dr. Suozzi recommends using nerve blocks for injecting HA filler or hyaluronidase in patients with scleroderma and minimizing the injection points. “Initially, we were using 30% lidocaine preparations around the mouth for an hour before the procedure, and patients were still having pain, so now we use nerve blocks,” she said. “For hyaluronidase, we do perform a test dose of 75-100 units, usually in the commissure. It’s amazing how well it works; people will usually come back after their test dose and have improvements in their measurements. This is a really easy treatment to perform, and I think it can be done in the office of a general dermatologist. There is concern about cross-reactivity with bee venom, so you want to ask patients about that.”
Dr. Suozzi reported having no relevant financial relationships.
A version of this article first appeared on Medscape.com.
CARLSBAD, CALIFORNIA — In 2003, researchers asked 303 patients with systemic sclerosis (scleroderma) what bothered them most about their disease from an aesthetic standpoint: Orofacial features, such as thin lips and mouth furrows, or non-facial features, such as fingertip ulceration and waxy changes to the skin.
Respondents expressed significant concern about specific orofacial features, including thin lips (73%), mouth furrows (80%), loss of facial lines (68%), and a smaller, tighter mouth (77%).
“Patients with systemic sclerosis may have loss of vermilion lip, microstomia, and perioral rhytids,” Kathleen Cook Suozzi, MD, who directs the Aesthetic Dermatology Program at Yale University School of Medicine, New Haven, Connecticut, said at the Controversies and Conversations in Laser and Cosmetic Surgery annual symposium. “How can we address these changes for our patients?”
Recent research has shown that hyaluronidase injections can help improve orofacial changes commonly experienced by patients with scleroderma. In 2019, researchers in Alabama reported the case of a 53-year-old woman treated with hyaluronidase for scleroderma-induced microstomia. After four visits over 7 months and a total hyaluronidase dose of 470 IU, the patient reported an improved Mouth Handicap in Systemic Sclerosis (MHISS) score (38 of 48); subjective improvement of symptoms, including greater ease in eating and undergoing dental treatment; and improved mouth closure.
In 2023, researchers published a cohort study of four women between the ages of 43 and 61 with autoimmune sclerosing conditions that resulted in oral microstomia. Following hyaluronidase injections, all improved in mouth opening capacity and MHISS, with change stabilizing between three and five treatments. More recently, in a study pending publication in JAAD Case Reports, Dr. Suozzi and colleagues retrospectively evaluated 12 women with scleroderma who received between 150 and 300 units of hyaluronic acid (HA) filler for microstomia between 2020 and 2023. Of the 12 women, 58% had diffuse disease, and 42% had limited disease. Overall, oral aperture width increased by 0.65 cm (P = .0027) and oral aperture height increased by 0.88 cm (P < .0001). “In general, patients needed three to four treatments to reach peak effect, and then they reached a plateau,” Dr. Suozzi said. “It wasn’t that the treatment wasn’t working anymore, but it was because their oral aperture had gotten to a size of around 5 cm, which is clinically normal. Interestingly, we found that if the patient’s disease flared and their microstomia started to return, when you rechallenged them, they continued to respond. So, patients can continue to use this treatment over time.”
In a separate case series of seven patients, Dr. Suozzi and colleagues prospectively evaluated the effect of HA soft tissue filler with Restylane Silk for lip augmentation. Study participants experienced statistically significant increases in the difference between pre- and postinjection fullness in both upper and lower lips. Also, the mean posttreatment score fell between “much improved” (2) and “improved” (3) on both the Investigator Global Aesthetic Improvement Scale and the Subject Global Aesthetic Improvement Scale.
Dr. Suozzi recommends using nerve blocks for injecting HA filler or hyaluronidase in patients with scleroderma and minimizing the injection points. “Initially, we were using 30% lidocaine preparations around the mouth for an hour before the procedure, and patients were still having pain, so now we use nerve blocks,” she said. “For hyaluronidase, we do perform a test dose of 75-100 units, usually in the commissure. It’s amazing how well it works; people will usually come back after their test dose and have improvements in their measurements. This is a really easy treatment to perform, and I think it can be done in the office of a general dermatologist. There is concern about cross-reactivity with bee venom, so you want to ask patients about that.”
Dr. Suozzi reported having no relevant financial relationships.
A version of this article first appeared on Medscape.com.
CARLSBAD, CALIFORNIA — In 2003, researchers asked 303 patients with systemic sclerosis (scleroderma) what bothered them most about their disease from an aesthetic standpoint: Orofacial features, such as thin lips and mouth furrows, or non-facial features, such as fingertip ulceration and waxy changes to the skin.
Respondents expressed significant concern about specific orofacial features, including thin lips (73%), mouth furrows (80%), loss of facial lines (68%), and a smaller, tighter mouth (77%).
“Patients with systemic sclerosis may have loss of vermilion lip, microstomia, and perioral rhytids,” Kathleen Cook Suozzi, MD, who directs the Aesthetic Dermatology Program at Yale University School of Medicine, New Haven, Connecticut, said at the Controversies and Conversations in Laser and Cosmetic Surgery annual symposium. “How can we address these changes for our patients?”
Recent research has shown that hyaluronidase injections can help improve orofacial changes commonly experienced by patients with scleroderma. In 2019, researchers in Alabama reported the case of a 53-year-old woman treated with hyaluronidase for scleroderma-induced microstomia. After four visits over 7 months and a total hyaluronidase dose of 470 IU, the patient reported an improved Mouth Handicap in Systemic Sclerosis (MHISS) score (38 of 48); subjective improvement of symptoms, including greater ease in eating and undergoing dental treatment; and improved mouth closure.
In 2023, researchers published a cohort study of four women between the ages of 43 and 61 with autoimmune sclerosing conditions that resulted in oral microstomia. Following hyaluronidase injections, all improved in mouth opening capacity and MHISS, with change stabilizing between three and five treatments. More recently, in a study pending publication in JAAD Case Reports, Dr. Suozzi and colleagues retrospectively evaluated 12 women with scleroderma who received between 150 and 300 units of hyaluronic acid (HA) filler for microstomia between 2020 and 2023. Of the 12 women, 58% had diffuse disease, and 42% had limited disease. Overall, oral aperture width increased by 0.65 cm (P = .0027) and oral aperture height increased by 0.88 cm (P < .0001). “In general, patients needed three to four treatments to reach peak effect, and then they reached a plateau,” Dr. Suozzi said. “It wasn’t that the treatment wasn’t working anymore, but it was because their oral aperture had gotten to a size of around 5 cm, which is clinically normal. Interestingly, we found that if the patient’s disease flared and their microstomia started to return, when you rechallenged them, they continued to respond. So, patients can continue to use this treatment over time.”
In a separate case series of seven patients, Dr. Suozzi and colleagues prospectively evaluated the effect of HA soft tissue filler with Restylane Silk for lip augmentation. Study participants experienced statistically significant increases in the difference between pre- and postinjection fullness in both upper and lower lips. Also, the mean posttreatment score fell between “much improved” (2) and “improved” (3) on both the Investigator Global Aesthetic Improvement Scale and the Subject Global Aesthetic Improvement Scale.
Dr. Suozzi recommends using nerve blocks for injecting HA filler or hyaluronidase in patients with scleroderma and minimizing the injection points. “Initially, we were using 30% lidocaine preparations around the mouth for an hour before the procedure, and patients were still having pain, so now we use nerve blocks,” she said. “For hyaluronidase, we do perform a test dose of 75-100 units, usually in the commissure. It’s amazing how well it works; people will usually come back after their test dose and have improvements in their measurements. This is a really easy treatment to perform, and I think it can be done in the office of a general dermatologist. There is concern about cross-reactivity with bee venom, so you want to ask patients about that.”
Dr. Suozzi reported having no relevant financial relationships.
A version of this article first appeared on Medscape.com.
When Does Different Types of Organ Damage From Lupus Occur? Long-Term Study Sheds Light
TOPLINE:
The first year after the diagnosis of systemic lupus erythematosus (SLE) is crucial, with the highest percentage of patients experiencing organ damage. Cardiovascular issues are the second most prevalent after musculoskeletal damage in both early and later stages of SLE.
METHODOLOGY:
- Researchers assessed organ damage persisting at least 6 months over different stages of lupus in 4219 patients with SLE (mean age, 35.9 years; 89.6% women) from the Spanish Society of Rheumatology Lupus Registry.
- Damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI).
- Longitudinal analysis was conducted globally and by each SDI domain on 1274 patients with recorded damage event dates.
- Follow-up data were available out to 10 years in 1113 patients and to 20 years in 601.
TAKEAWAY:
- New damage was recorded in 20% of the patients with SLE within the first year after diagnosis, with the annual percentage of patients with new damage decreasing to 5% after the first 5 years of follow-up.
- In the first year, musculoskeletal damage was reported by the highest proportion of patients (21%), followed by cardiovascular damage inclusive of cerebrovascular accidents and claudication for 6 months (19%).
- The cardiovascular system remained the second most affected system even during the later stages of the diseases at years 10 and 20 of follow-up (20%-25%).
- Apart from musculoskeletal and cardiovascular damage, patients with lupus also showed renal and ocular damage in the early and later stages of the disease, respectively.
IN PRACTICE:
“Our study highlights the importance of cardiovascular damage and the need for its prevention during the earliest stages of the disease,” the authors wrote.
SOURCE:
The study was led by Irene Altabás-González, MD, PhD, Rheumatology Department, Vigo University Hospital Group, Vigo, Spain. It was published online in Lupus Science & Medicine.
LIMITATIONS:
The retrospective collection of data in the study may have led to missing items; for example, the dates of damage events for the whole cohort were not available.
DISCLOSURES:
The registry was supported by the Spanish Society of Rheumatology. No specific funding was received for the study. The authors declared no conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
A version of this article appeared on Medscape.com.
TOPLINE:
The first year after the diagnosis of systemic lupus erythematosus (SLE) is crucial, with the highest percentage of patients experiencing organ damage. Cardiovascular issues are the second most prevalent after musculoskeletal damage in both early and later stages of SLE.
METHODOLOGY:
- Researchers assessed organ damage persisting at least 6 months over different stages of lupus in 4219 patients with SLE (mean age, 35.9 years; 89.6% women) from the Spanish Society of Rheumatology Lupus Registry.
- Damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI).
- Longitudinal analysis was conducted globally and by each SDI domain on 1274 patients with recorded damage event dates.
- Follow-up data were available out to 10 years in 1113 patients and to 20 years in 601.
TAKEAWAY:
- New damage was recorded in 20% of the patients with SLE within the first year after diagnosis, with the annual percentage of patients with new damage decreasing to 5% after the first 5 years of follow-up.
- In the first year, musculoskeletal damage was reported by the highest proportion of patients (21%), followed by cardiovascular damage inclusive of cerebrovascular accidents and claudication for 6 months (19%).
- The cardiovascular system remained the second most affected system even during the later stages of the diseases at years 10 and 20 of follow-up (20%-25%).
- Apart from musculoskeletal and cardiovascular damage, patients with lupus also showed renal and ocular damage in the early and later stages of the disease, respectively.
IN PRACTICE:
“Our study highlights the importance of cardiovascular damage and the need for its prevention during the earliest stages of the disease,” the authors wrote.
SOURCE:
The study was led by Irene Altabás-González, MD, PhD, Rheumatology Department, Vigo University Hospital Group, Vigo, Spain. It was published online in Lupus Science & Medicine.
LIMITATIONS:
The retrospective collection of data in the study may have led to missing items; for example, the dates of damage events for the whole cohort were not available.
DISCLOSURES:
The registry was supported by the Spanish Society of Rheumatology. No specific funding was received for the study. The authors declared no conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
A version of this article appeared on Medscape.com.
TOPLINE:
The first year after the diagnosis of systemic lupus erythematosus (SLE) is crucial, with the highest percentage of patients experiencing organ damage. Cardiovascular issues are the second most prevalent after musculoskeletal damage in both early and later stages of SLE.
METHODOLOGY:
- Researchers assessed organ damage persisting at least 6 months over different stages of lupus in 4219 patients with SLE (mean age, 35.9 years; 89.6% women) from the Spanish Society of Rheumatology Lupus Registry.
- Damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI).
- Longitudinal analysis was conducted globally and by each SDI domain on 1274 patients with recorded damage event dates.
- Follow-up data were available out to 10 years in 1113 patients and to 20 years in 601.
TAKEAWAY:
- New damage was recorded in 20% of the patients with SLE within the first year after diagnosis, with the annual percentage of patients with new damage decreasing to 5% after the first 5 years of follow-up.
- In the first year, musculoskeletal damage was reported by the highest proportion of patients (21%), followed by cardiovascular damage inclusive of cerebrovascular accidents and claudication for 6 months (19%).
- The cardiovascular system remained the second most affected system even during the later stages of the diseases at years 10 and 20 of follow-up (20%-25%).
- Apart from musculoskeletal and cardiovascular damage, patients with lupus also showed renal and ocular damage in the early and later stages of the disease, respectively.
IN PRACTICE:
“Our study highlights the importance of cardiovascular damage and the need for its prevention during the earliest stages of the disease,” the authors wrote.
SOURCE:
The study was led by Irene Altabás-González, MD, PhD, Rheumatology Department, Vigo University Hospital Group, Vigo, Spain. It was published online in Lupus Science & Medicine.
LIMITATIONS:
The retrospective collection of data in the study may have led to missing items; for example, the dates of damage events for the whole cohort were not available.
DISCLOSURES:
The registry was supported by the Spanish Society of Rheumatology. No specific funding was received for the study. The authors declared no conflicts of interest.
This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.
A version of this article appeared on Medscape.com.
Saxophone Penis: A Forgotten Manifestation of Hidradenitis Suppurativa
To the Editor:
Hidradenitis suppurativa (HS) is a multifactorial chronic inflammatory skin disease affecting 1% to 4% of Europeans. It is characterized by recurrent inflamed nodules, abscesses, and sinus tracts in intertriginous regions.1 The genital area is affected in 11% of cases2 and usually is connected to severe forms of HS in both men and women.3 The prevalence of HS-associated genital lymphedema remains unknown.
Saxophone penis is a specific penile malformation characterized by a saxophone shape due to inflammation of the major penile lymphatic vessels that cause fibrosis of the surrounding connective tissue. Poor blood flow further causes contracture and distortion of the penile axis.4 Saxophone penis also has been associated with primary lymphedema, lymphogranuloma venereum, filariasis,5 and administration of paraffin injections.6 We describe 3 men with HS who presented with saxophone penis.
A 33-year-old man with Hurley stage III HS presented with a medical history of groin lesions and progressive penoscrotal edema of 13 years’ duration. He had a body mass index (BMI) of 37, no family history of HS or comorbidities, and a 15-year history of smoking 20 cigarettes per day. After repeated surgical drainage of the HS lesions as well as antibiotic treatment with clindamycin 600 mg/d and rifampicin 600 mg/d, the patient was kept on a maintenance therapy with adalimumab 40 mg/wk. Due to lack of response, treatment was discontinued at week 16. Clindamycin and rifampicin 300 mg were immediately reintroduced with no benefit on the genital lesions. The patient underwent genital reconstruction, including penile degloving, scrotoplasty, infrapubic fat pad removal, and perineoplasty (Figure 1). The patient currently is not undergoing any therapies.
A 55-year-old man presented with Hurley stage II HS of 33 years’ duration. He had a BMI of 52; a history of hypertension, hyperuricemia, severe hip and knee osteoarthritis, and orchiopexy in childhood; a smoking history of 40 cigarettes per day; and an alcohol consumption history of 200 mL per day since 18 years of age. He had radical excision of axillary lesions 8 years prior. One year later, he was treated with concomitant clindamycin and rifampicin 300 mg twice daily for 3 months with no desirable effects. Adalimumab 40 mg/wk was initiated. After 12 weeks of treatment, he experienced 80% improvement in all areas except the genital region. He continued adalimumab for 3 years with good clinical response in all HS-affected sites except the genital region.
A 66-year-old man presented with Hurley stage III HS of 37 years’ duration. He had a smoking history of 10 cigarettes per day for 30 years, a BMI of 24.6, and a medical history of long-standing hypertension and hypothyroidism. A 3-month course of clindamycin and rifampicin 600 mg/d was ineffective; adalimumab 40 mg/wk was initiated. All affected areas improved, except for the saxophone penis. He continues his fifth year of therapy with adalimumab (Figure 2).
Hidradenitis suppurativa is associated with chronic pain, purulent malodor, and scarring with structural deformity. Repetitive inflammation causes fibrosis, scar formation, and soft-tissue destruction of lymphatic vessels, leading to lymphedema; primary lymphedema of the genitals in men has been reported to result in a saxophone penis.4
The only approved biologic treatments for moderate to severe HS are the tumor necrosis factor α inhibitor adalimumab and anti-IL-17 secukinumab.1 All 3 of our patients with HS were treated with adalimumab with reasonable success; however, the penile condition remained refractory, which we speculate may be due to adalimumab’s ability to control only active inflammatory lesions but not scars or fibrotic tissue.7 Higher adalimumab dosages were unlikely to be beneficial for their penile condition; some improvements have been reported following fluoroquinolone therapy. To our knowledge, there is no effective medical treatment for saxophone penis. However, surgery showed good results in one of our patients. Among our 3 adalimumab-treated patients, only 1 patient had corrective surgery that resulted in improvement in the penile deformity, further confirming adalimumab’s limited role in genital lymphedema.7 Extensive resection of the lymphedematous tissue, scrotoplasty, and Charles procedure are treatment options.8
Genital lymphedema has been associated with lymphangiectasia, lymphangioma circumscriptum, infections, and neoplasms such as lymphangiosarcoma and squamous cell carcinoma.9 Our patients reported discomfort, hygiene issues, and swelling. One patient reported micturition, and 2 patients reported sexual dysfunction.
Saxophone penis remains a disabling sequela of HS. Early diagnosis and treatment of HS may help prevent development of this condition.
- Lee EY, Alhusayen R, Lansang P, et al. What is hidradenitis suppurativa? Can Fam Physician. 2017;63:114-120.
- Fertitta L, Hotz C, Wolkenstein P, et al. Efficacy and satisfaction of surgical treatment for hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2020;34:839-845.
- Micieli R, Alavi A. Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature. Int J Dermatol. 2018;57:1471-1480.
- Maatouk I, Moutran R. Saxophone penis. JAMA Dermatol. 2013;149:802.
- Koley S, Mandal RK. Saxophone penis after unilateral inguinal bubo of lymphogranuloma venereum. Indian J Sex Transm Dis AIDS. 2013;34:149-151.
- D’Antuono A, Lambertini M, Gaspari V, et al. Visual dermatology: self-induced chronic saxophone penis due to paraffin injections. J Cutan Med Surg. 2019;23:330.
- Musumeci ML, Scilletta A, Sorci F, et al. Genital lymphedema associated with hidradenitis suppurativa unresponsive to adalimumab treatment. JAAD Case Rep. 2019;5:326-328.
- Jain V, Singh S, Garge S, et al. Saxophone penis due to primary lymphoedema. J Indian Assoc Pediatr Surg. 2009;14:230-231.
- Moosbrugger EA, Mutasim DF. Hidradenitis suppurativa complicated by severe lymphedema and lymphangiectasias. J Am Acad Dermatol. 2011;64:1223-1224.
To the Editor:
Hidradenitis suppurativa (HS) is a multifactorial chronic inflammatory skin disease affecting 1% to 4% of Europeans. It is characterized by recurrent inflamed nodules, abscesses, and sinus tracts in intertriginous regions.1 The genital area is affected in 11% of cases2 and usually is connected to severe forms of HS in both men and women.3 The prevalence of HS-associated genital lymphedema remains unknown.
Saxophone penis is a specific penile malformation characterized by a saxophone shape due to inflammation of the major penile lymphatic vessels that cause fibrosis of the surrounding connective tissue. Poor blood flow further causes contracture and distortion of the penile axis.4 Saxophone penis also has been associated with primary lymphedema, lymphogranuloma venereum, filariasis,5 and administration of paraffin injections.6 We describe 3 men with HS who presented with saxophone penis.
A 33-year-old man with Hurley stage III HS presented with a medical history of groin lesions and progressive penoscrotal edema of 13 years’ duration. He had a body mass index (BMI) of 37, no family history of HS or comorbidities, and a 15-year history of smoking 20 cigarettes per day. After repeated surgical drainage of the HS lesions as well as antibiotic treatment with clindamycin 600 mg/d and rifampicin 600 mg/d, the patient was kept on a maintenance therapy with adalimumab 40 mg/wk. Due to lack of response, treatment was discontinued at week 16. Clindamycin and rifampicin 300 mg were immediately reintroduced with no benefit on the genital lesions. The patient underwent genital reconstruction, including penile degloving, scrotoplasty, infrapubic fat pad removal, and perineoplasty (Figure 1). The patient currently is not undergoing any therapies.
A 55-year-old man presented with Hurley stage II HS of 33 years’ duration. He had a BMI of 52; a history of hypertension, hyperuricemia, severe hip and knee osteoarthritis, and orchiopexy in childhood; a smoking history of 40 cigarettes per day; and an alcohol consumption history of 200 mL per day since 18 years of age. He had radical excision of axillary lesions 8 years prior. One year later, he was treated with concomitant clindamycin and rifampicin 300 mg twice daily for 3 months with no desirable effects. Adalimumab 40 mg/wk was initiated. After 12 weeks of treatment, he experienced 80% improvement in all areas except the genital region. He continued adalimumab for 3 years with good clinical response in all HS-affected sites except the genital region.
A 66-year-old man presented with Hurley stage III HS of 37 years’ duration. He had a smoking history of 10 cigarettes per day for 30 years, a BMI of 24.6, and a medical history of long-standing hypertension and hypothyroidism. A 3-month course of clindamycin and rifampicin 600 mg/d was ineffective; adalimumab 40 mg/wk was initiated. All affected areas improved, except for the saxophone penis. He continues his fifth year of therapy with adalimumab (Figure 2).
Hidradenitis suppurativa is associated with chronic pain, purulent malodor, and scarring with structural deformity. Repetitive inflammation causes fibrosis, scar formation, and soft-tissue destruction of lymphatic vessels, leading to lymphedema; primary lymphedema of the genitals in men has been reported to result in a saxophone penis.4
The only approved biologic treatments for moderate to severe HS are the tumor necrosis factor α inhibitor adalimumab and anti-IL-17 secukinumab.1 All 3 of our patients with HS were treated with adalimumab with reasonable success; however, the penile condition remained refractory, which we speculate may be due to adalimumab’s ability to control only active inflammatory lesions but not scars or fibrotic tissue.7 Higher adalimumab dosages were unlikely to be beneficial for their penile condition; some improvements have been reported following fluoroquinolone therapy. To our knowledge, there is no effective medical treatment for saxophone penis. However, surgery showed good results in one of our patients. Among our 3 adalimumab-treated patients, only 1 patient had corrective surgery that resulted in improvement in the penile deformity, further confirming adalimumab’s limited role in genital lymphedema.7 Extensive resection of the lymphedematous tissue, scrotoplasty, and Charles procedure are treatment options.8
Genital lymphedema has been associated with lymphangiectasia, lymphangioma circumscriptum, infections, and neoplasms such as lymphangiosarcoma and squamous cell carcinoma.9 Our patients reported discomfort, hygiene issues, and swelling. One patient reported micturition, and 2 patients reported sexual dysfunction.
Saxophone penis remains a disabling sequela of HS. Early diagnosis and treatment of HS may help prevent development of this condition.
To the Editor:
Hidradenitis suppurativa (HS) is a multifactorial chronic inflammatory skin disease affecting 1% to 4% of Europeans. It is characterized by recurrent inflamed nodules, abscesses, and sinus tracts in intertriginous regions.1 The genital area is affected in 11% of cases2 and usually is connected to severe forms of HS in both men and women.3 The prevalence of HS-associated genital lymphedema remains unknown.
Saxophone penis is a specific penile malformation characterized by a saxophone shape due to inflammation of the major penile lymphatic vessels that cause fibrosis of the surrounding connective tissue. Poor blood flow further causes contracture and distortion of the penile axis.4 Saxophone penis also has been associated with primary lymphedema, lymphogranuloma venereum, filariasis,5 and administration of paraffin injections.6 We describe 3 men with HS who presented with saxophone penis.
A 33-year-old man with Hurley stage III HS presented with a medical history of groin lesions and progressive penoscrotal edema of 13 years’ duration. He had a body mass index (BMI) of 37, no family history of HS or comorbidities, and a 15-year history of smoking 20 cigarettes per day. After repeated surgical drainage of the HS lesions as well as antibiotic treatment with clindamycin 600 mg/d and rifampicin 600 mg/d, the patient was kept on a maintenance therapy with adalimumab 40 mg/wk. Due to lack of response, treatment was discontinued at week 16. Clindamycin and rifampicin 300 mg were immediately reintroduced with no benefit on the genital lesions. The patient underwent genital reconstruction, including penile degloving, scrotoplasty, infrapubic fat pad removal, and perineoplasty (Figure 1). The patient currently is not undergoing any therapies.
A 55-year-old man presented with Hurley stage II HS of 33 years’ duration. He had a BMI of 52; a history of hypertension, hyperuricemia, severe hip and knee osteoarthritis, and orchiopexy in childhood; a smoking history of 40 cigarettes per day; and an alcohol consumption history of 200 mL per day since 18 years of age. He had radical excision of axillary lesions 8 years prior. One year later, he was treated with concomitant clindamycin and rifampicin 300 mg twice daily for 3 months with no desirable effects. Adalimumab 40 mg/wk was initiated. After 12 weeks of treatment, he experienced 80% improvement in all areas except the genital region. He continued adalimumab for 3 years with good clinical response in all HS-affected sites except the genital region.
A 66-year-old man presented with Hurley stage III HS of 37 years’ duration. He had a smoking history of 10 cigarettes per day for 30 years, a BMI of 24.6, and a medical history of long-standing hypertension and hypothyroidism. A 3-month course of clindamycin and rifampicin 600 mg/d was ineffective; adalimumab 40 mg/wk was initiated. All affected areas improved, except for the saxophone penis. He continues his fifth year of therapy with adalimumab (Figure 2).
Hidradenitis suppurativa is associated with chronic pain, purulent malodor, and scarring with structural deformity. Repetitive inflammation causes fibrosis, scar formation, and soft-tissue destruction of lymphatic vessels, leading to lymphedema; primary lymphedema of the genitals in men has been reported to result in a saxophone penis.4
The only approved biologic treatments for moderate to severe HS are the tumor necrosis factor α inhibitor adalimumab and anti-IL-17 secukinumab.1 All 3 of our patients with HS were treated with adalimumab with reasonable success; however, the penile condition remained refractory, which we speculate may be due to adalimumab’s ability to control only active inflammatory lesions but not scars or fibrotic tissue.7 Higher adalimumab dosages were unlikely to be beneficial for their penile condition; some improvements have been reported following fluoroquinolone therapy. To our knowledge, there is no effective medical treatment for saxophone penis. However, surgery showed good results in one of our patients. Among our 3 adalimumab-treated patients, only 1 patient had corrective surgery that resulted in improvement in the penile deformity, further confirming adalimumab’s limited role in genital lymphedema.7 Extensive resection of the lymphedematous tissue, scrotoplasty, and Charles procedure are treatment options.8
Genital lymphedema has been associated with lymphangiectasia, lymphangioma circumscriptum, infections, and neoplasms such as lymphangiosarcoma and squamous cell carcinoma.9 Our patients reported discomfort, hygiene issues, and swelling. One patient reported micturition, and 2 patients reported sexual dysfunction.
Saxophone penis remains a disabling sequela of HS. Early diagnosis and treatment of HS may help prevent development of this condition.
- Lee EY, Alhusayen R, Lansang P, et al. What is hidradenitis suppurativa? Can Fam Physician. 2017;63:114-120.
- Fertitta L, Hotz C, Wolkenstein P, et al. Efficacy and satisfaction of surgical treatment for hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2020;34:839-845.
- Micieli R, Alavi A. Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature. Int J Dermatol. 2018;57:1471-1480.
- Maatouk I, Moutran R. Saxophone penis. JAMA Dermatol. 2013;149:802.
- Koley S, Mandal RK. Saxophone penis after unilateral inguinal bubo of lymphogranuloma venereum. Indian J Sex Transm Dis AIDS. 2013;34:149-151.
- D’Antuono A, Lambertini M, Gaspari V, et al. Visual dermatology: self-induced chronic saxophone penis due to paraffin injections. J Cutan Med Surg. 2019;23:330.
- Musumeci ML, Scilletta A, Sorci F, et al. Genital lymphedema associated with hidradenitis suppurativa unresponsive to adalimumab treatment. JAAD Case Rep. 2019;5:326-328.
- Jain V, Singh S, Garge S, et al. Saxophone penis due to primary lymphoedema. J Indian Assoc Pediatr Surg. 2009;14:230-231.
- Moosbrugger EA, Mutasim DF. Hidradenitis suppurativa complicated by severe lymphedema and lymphangiectasias. J Am Acad Dermatol. 2011;64:1223-1224.
- Lee EY, Alhusayen R, Lansang P, et al. What is hidradenitis suppurativa? Can Fam Physician. 2017;63:114-120.
- Fertitta L, Hotz C, Wolkenstein P, et al. Efficacy and satisfaction of surgical treatment for hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2020;34:839-845.
- Micieli R, Alavi A. Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature. Int J Dermatol. 2018;57:1471-1480.
- Maatouk I, Moutran R. Saxophone penis. JAMA Dermatol. 2013;149:802.
- Koley S, Mandal RK. Saxophone penis after unilateral inguinal bubo of lymphogranuloma venereum. Indian J Sex Transm Dis AIDS. 2013;34:149-151.
- D’Antuono A, Lambertini M, Gaspari V, et al. Visual dermatology: self-induced chronic saxophone penis due to paraffin injections. J Cutan Med Surg. 2019;23:330.
- Musumeci ML, Scilletta A, Sorci F, et al. Genital lymphedema associated with hidradenitis suppurativa unresponsive to adalimumab treatment. JAAD Case Rep. 2019;5:326-328.
- Jain V, Singh S, Garge S, et al. Saxophone penis due to primary lymphoedema. J Indian Assoc Pediatr Surg. 2009;14:230-231.
- Moosbrugger EA, Mutasim DF. Hidradenitis suppurativa complicated by severe lymphedema and lymphangiectasias. J Am Acad Dermatol. 2011;64:1223-1224.
Practice Points
- Hidradenitis suppurativa (HS) is a multifactorial chronic inflammatory skin disease.
- Saxophone penis is a specific penile malformation characterized by a saxophone shape due to inflammation.
- Repetitive inflammation within the context of HS may cause structural deformity of the penis, resulting in a saxophone penis.
- Early diagnosis and treatment of HS may help prevent development of this condition.