All Publications

  • Association Between Autoantibody Phenotype and Cutaneous Adverse Reactions to Hydroxychloroquine in Dermatomyositis. JAMA dermatology Wolstencroft, P. W., Casciola-Rosen, L., Fiorentino, D. F. 2018


    Importance: Hydroxychloroquine sulfate is a commonly used medication for patients with dermatomyositis and has been associated with a uniquely elevated risk of adverse cutaneous reactions in this population. No studies to date have examined whether certain subsets of patients with dermatomyositis are at increased risk of experiencing a hydroxychloroquine-associated skin eruption.Objective: To identify disease features that increase the risk of hydroxychloroquine-associated skin eruption in adults with dermatomyositis.Design, Setting, and Participants: A retrospective cohort study was conducted in the outpatient dermatology clinic at a tertiary academic referral center. All adults with dermatomyositis (age >18 years) who started receiving hydroxychloroquine between July 1, 1990, and September 13, 2016, were eligible for the analysis. Patients were considered to have a hydroxychloroquine-associated skin eruption if a skin eruption had developed within their first 4 weeks of treatment and resolved with discontinuation of hydroxychloroquine therapy.Exposures: One or more doses of hydroxychloroquine.Main Outcomes and Measures: The associations between autoantibodies (against transcription intermediary factor 1gamma [TIF-1gamma], nucleosome-remodeling deacetylase complex [Mi-2], nuclear matrix protein [NXP-2], small ubiquitinlike modifier 1 activating enzyme [SAE-1/2], melanoma differentiation-associated gene 5 [MDA-5], histidyl-transfer RNA synthetase [Jo-1], Ku, and signal recognition particles) and cutaneous adverse reactions to hydroxychloroquine in patients with dermatomyositis.Results: A total of 111 patients met the inclusion criteria, and 23 (20.7%) developed a hydroxychloroquine-associated skin eruption (20 [87.0%] were women with a mean [SD] age of 49 [14] years at diagnosis). Skin eruptions were approximately 3 times more common in patients with anti-SAE-1/2 autoantibodies (7 of 14 [50.0%]) compared with those without the autoantibody (16 of 97 [16.5%]). In contrast, none of 15 patients with anti-MDA-5 autoantibodies had a skin eruption vs 23 of 96 (24.0%) of those without the autoantibody. In exact logistic regressions adjusted for age, race/ethnicity, sex, amyopathic status, anti-Ro52 status, and dermatomyositis-associated cancer, the presence of anti-SAE-1/2 autoantibodies was significantly associated with a hydroxychloroquine-associated skin eruption (odds ratio [OR], 8.43; 95% CI, 1.98-49.19; P=.003) and presence of anti-MDA-5 autoantibodies was significantly negatively associated with a hydroxychloroquine-associated skin eruption (OR, 0.06; 95% CI, 0.0004-0.52; P=.006). No other autoantibodies were significantly positively or negatively associated with a hydroxychloroquine-associated skin eruption.Conclusions and Relevance: Adverse skin reactions to hydroxychloroquine are relatively common in a US cohort of patients with dermatomyositis. Our data suggest that pathophysiologic differences exist between autoantibody subsets in dermatomyositis.

    View details for PubMedID 30140893

  • Dermatomyositis Clinical and Pathological Phenotypes Associated with Myositis-Specific Autoantibodies Current Rheumatology Reports Wolstencroft, P. W., Fiorentino, D. F. 2018; 20 (5)
  • Factors Associated With Clinical Remission of Skin Disease in Dermatomyositis JAMA DERMATOLOGY Wolstencroft, P. W., Chung, L., Li, S., Casciola-Rosen, L., Fiorentino, D. F. 2018; 154 (1): 44?51


    Cutaneous disease represents a significant burden for patients with dermatomyositis. However, quantitative estimates of the probability of skin disease remission and clinical factors associated with skin outcomes are lacking.To characterize cutaneous disease course in adult patients with dermatomyositis.Prospective cohort study conducted at a dermatology clinic at a tertiary academic referral center. All adult patients with dermatomyositis (age?>18 years) seen between May 15, 2007, and October 28, 2016, were eligible. Patients were included in the current analysis if they had a baseline Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity score of 12 or higher, and 2 or more CDASI scores separated by 3 months or more within their first 3 years of follow-up.The percentage of patients who achieved clinical remission of their cutaneous disease as measured by the CDASI over a 3-year follow-up.A total of 74 patients met our inclusion criteria (mean [SD] age at initial CDASI scoring, 54 [13] years; 58 women [78%]), and 28 (38%) achieved clinical remission during our 3-year follow-up period. Increased age (odds ratio [OR], 1.07; 95% CI, 1.02-1.12; P?=?.01), a dermatomyositis-associated malignancy (OR, 14.46; 95% CI, 2.18-96.07; P?=?.01), and treatment with mycophenolate mofetil (OR, 6.00; 95% CI, 1.66-21.78; P?=?.01) were significantly associated with clinical remission of skin disease in multivariable analysis. Patients with anti-melanoma differentiation-associated protein 5 antibodies had a significantly lower probability of meeting outcome criteria in our time-to-event analysis. Baseline cutaneous disease activity, disease duration at baseline, and disease duration before first systemic therapy were not significantly associated with clinical remission of skin disease.Clinical remission was relatively uncommon in our population despite aggressive systemic therapy, and patients with anti-melanoma differentiation-associated protein 5 antibodies were even less likely to enter clinical remission during a 3-year follow-up period. Although mycophenolate mofetil compared favorably with other treatment options, our data provide evidence that a substantial population of patients with dermatomyositis have skin disease that is not adequately managed with standard-of-care therapies.

    View details for PubMedID 29114741

    View details for PubMedCentralID PMC5833585

  • Factors Associated with Clinical Remission of Skin Disease in Dermatomyositis Wolstencroft, P., Chung, L., Li, S., Casciola-Rosen, L., Fiorentino, D. WILEY. 2017
  • Verbigeration: An overlooked symptom of a "forgotten syndrome"? Bipolar disorders Mason, D. P., Tan, M., Lee, J., Wolstencroft, P., Sanborn, K., Ballon, J. S. 2017; 19 (8): 710?12

    View details for PubMedID 29268005

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