Peter B Chansky1, Jeannette M Olazagasti2, Rui Feng3, Victoria P Werth4. 1. Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania; Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. 2. Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania; Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania; University of Texas Southwestern Medical Center, Dallas, Texas. 3. Department of Biostatistics and Epidemiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. 4. Corporal Michael J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania; Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address: werth@pennmedicine.upenn.edu.
Abstract
BACKGROUND: Limited studies describe the longitudinal course of cutaneous dermatomyositis (DM). OBJECTIVE: To characterize disease course in treated cutaneous DM by using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a validated outcome instrument. METHODS: A retrospective cohort included patients with DM who had their CDASI activity subscore recorded for at least 2 years. Disease progression was classified as improved, worsened, or stable, and disease course was classified as monophasic, polyphasic, or chronic. Subjects were divided into groups with disease of mild (n = 16) and moderate-to-severe (n = 24) baseline severity. RESULTS: A total of 40 patients with DM met inclusion criteria. The majority demonstrated improvement in disease activity (n = 21 [52.5%]) rather than worsening (n = 7 [17.5%]) and stable (n = 12 [30.0%]) disease. Most patients with mild disease remained stable (n = 10 [62.5%]), whereas most with moderate-to-severe disease improved (n = 19 [79.2%]). A polyphasic course (n = 33 [82.5%]) predominated over monophasic (n = 5 [12.5%]) and chronic (n = 2 [5%]) courses. The average number of flares per year per number of years of follow-up was independent of baseline disease activity. LIMITATIONS: The retrospective design, potential referral bias, and cutoff values in classification criteria are limitations. CONCLUSION: Baseline CDASI activity score is associated with particular patterns of disease course and progression in cutaneous DM. Published by Elsevier Inc.
BACKGROUND: Limited studies describe the longitudinal course of cutaneous dermatomyositis (DM). OBJECTIVE: To characterize disease course in treated cutaneous DM by using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a validated outcome instrument. METHODS: A retrospective cohort included patients with DM who had their CDASI activity subscore recorded for at least 2 years. Disease progression was classified as improved, worsened, or stable, and disease course was classified as monophasic, polyphasic, or chronic. Subjects were divided into groups with disease of mild (n = 16) and moderate-to-severe (n = 24) baseline severity. RESULTS: A total of 40 patients with DM met inclusion criteria. The majority demonstrated improvement in disease activity (n = 21 [52.5%]) rather than worsening (n = 7 [17.5%]) and stable (n = 12 [30.0%]) disease. Most patients with mild disease remained stable (n = 10 [62.5%]), whereas most with moderate-to-severe disease improved (n = 19 [79.2%]). A polyphasic course (n = 33 [82.5%]) predominated over monophasic (n = 5 [12.5%]) and chronic (n = 2 [5%]) courses. The average number of flares per year per number of years of follow-up was independent of baseline disease activity. LIMITATIONS: The retrospective design, potential referral bias, and cutoff values in classification criteria are limitations. CONCLUSION: Baseline CDASI activity score is associated with particular patterns of disease course and progression in cutaneous DM. Published by Elsevier Inc.
Entities:
Keywords:
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI); cutaneous; dermatomyositis; disease activity; disease course; longitudinal; skin predominant
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