Cutaneous ulcers in anti-MDA5-positive dermatomyositis with rapidly progressive interstitial lung disease: a multicentre retrospective cohort study

Abstract

IntroductionTo identify the clinical characteristics and risk factors for cutaneous ulcers in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM) combined with rapidly progressive interstitial lung disease (RPILD).Material and methodsWe conducted a retrospective cohort study on the medical records of patients enrolled from the Nanjing Medical University Myositis-associated ILD cohort (NMMI). The clinical characteristics of patients in the ulcer-positive group were compared with those in the ulcer-negative group by chi-square or Fisher’s exact test. Univariate and multivariate logistic regression analyses were used to assess risk factors for the development of cutaneous ulcers.ResultsA total of 246 patients with anti-MDA5+ DM were retrospectively enrolled in the study, including 176 females (176/246, 71.54%) and 70 males (70/246, 28.46%), with a female-to-male ratio of 2.51 : 1. Among the 246 patients, a total of 88 cases (88/246, 35.77%) with anti-MDA5+ DM combined with RPILD were further studied, including 55 females (55/88, 62.5%) and 33 males (33/88, 37.5%), with a female-to-male ratio of 1.67 : 1. Twelve patients (12/88, 13.64%) had cutaneous ulcers. In terms of clinical characteristics, patients in the ulcer-positive group had significantly more proximal muscle involvement (83.33% vs. 38.16%, p = 0.003) and more heliotrope rash (83.33% vs. 43.42%, p = 0.010) than in the ulcer-negative group. In univariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 8.103; 95% CI: 1.657–39.625; p = 0.010) and heliotrope rash (OR = 6.515; 95% CI: 1.336–31.773; p = 0.020). In multivariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 6.436; 95% CI: 1.274–32.524; p = 0.024), and proximal muscle involvement was an independent risk factor for cutaneous ulcers.ConclusionsWe confirmed the association between cutaneous ulcers and proximal muscle involvement and heliotrope rash in patients with anti-MDA5+ DM combined with RPILD. Proximal muscle involvement is an independent risk factor for cutaneous ulcers.