Factors Associated With Clinical Remission of Skin Disease in Dermatomyositis

Abstract

IMPORTANCE 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.OBJECTIVE To characterize cutaneous disease course in adult patients with dermatomyositis. DESIGN, SETTING, AND PARTICIPANTS 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. MAIN OUTCOMES AND MEASURESThe percentage of patients who achieved clinical remission of their cutaneous disease as measured by the CDASI over a 3-year follow-up.RESULTS 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.CONCLUSIONS AND RELEVANCE Clinical remission was relatively uncommon in our population despite aggressive systemic therapy, and patients with anti-melanoma differentiationassociated 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.

Anti-TIF1gamma Antibody-Positive Dermatomyositis Associated with Myelodysplastic Syndrome: Response to Treatment

“…Anti-TIF1gamma antibody-positive DM usually presents with extensive cutaneous disease, but only mild muscle weakness; cutaneous findings more specific to anti-TIFgamma antibody-positive DM include red-on-white poikiloderma of the scalp and extensive psoriasiform lesions - features that were prominent in our patient (Figure 1) [2,11]. Recent data suggests that most patients who achieve a clinical response of malignancy also achieve a clinical response of DM [12]. Moreover, factors that positively correlate with clinical response of DM include increased age and malignancy [12].…”

IL18-containing 5-gene signature distinguishes histologically identical dermatomyositis and lupus erythematosus skin lesions

“…Melanoma differentiation-associated protein 5 (MDA5) antibody–positive (MDA5 + ) patients had the highest serum IFN levels when compared with DM patients with anti–aminoacyl-tRNA synthetase antibodies or no autoantibody positivity ( 13 ). Interestingly, anti-MDA5 antibodies were also associated with the most refractory skin findings in 1 cohort of 74 patients ( 12 ). Our cohort, however, did not have sufficient MDA5 + patient numbers to analyze correlation with IFN in the skin.…”

“…DM skin lesions can be challenging to treat ( 12 ) and may be difficult to distinguish from cutaneous lupus lesions by biopsy. This study used a large cohort of DM skin lesions to identify transcriptional changes in the skin to generate a better understanding of disease pathology and to provide clues for improved treatment of refractory skin lesions.…”

Hypomyopathic Dermatomyositis with Refractory Dermatitis Treated by Low-dose IL-2

“…Most patients with skin disease respond to the initial therapy, and some achieve sustained disease control either off all therapy or with low-dose maintenance therapy. Patients who fail to respond to conventional interventions or who relapse after an initial response have refractory disease and require the initiation of more aggressive therapies [4,5]. Here, we describe a patient with hypomyopathic DM with refractive dermatitis.…”