Fever, Cutaneous Ulcers, and Arthritis Define the MDA5 Phenotype in Indian Patients with Idiopathic Inflammatory Myositis.

Abstract

In idiopathic inflammatory myositis (IIM), anti-MDA5 (melanoma differentiation-associated gene 5) antibody-associated DM (MDA5 DM) is a distinct subset characterised by presentation with cutaneous involvement, association with ILD and delayed onset of muscle involvement which is believed to be not severe. To study the clinical profile and treatment outcomes of MDA5 DM patients. Records of patients fulfilling the ACR/EULAR classification criteria for IIM and testing positive for MDA5 antibody, were retrieved from the ongoingMyoIN registry database of our centre. Clinical, laboratory and treatment data were analysed. Follow-up details were noted. Parameters were compared between survivors and non-survivors using student's t-test or Mann-Whitney U tests for continuous variables and Chi square test and Fisher's exact for categorical variables. Eighteen patients (5 juvenile) were identified. Median age was 30(16-41) years and disease duration at time of diagnosis was 5.5 months with median follow up duration of 18 months (12-31). The prevalence of constitutional symptoms, cutaneous involvement, arthritis, and myositis were 94%, 100%, 78%, and 44% respectively. Ulcers were the most common cutaneous finding. Myositis when present was severe. Interstitial lung disease (ILD) was present in 6 patients which was rapidly progressive (RPILD) in 3. Anti-Ro-52 antibody (n=5) was the most common myositis associated antibody (MAA). There was no difference in the clinical profile of children and adults. Seven patients succumbed. RPILD was a predictor of mortality (p=0.04). Cutaneous relapses were common among survivors but responded well to further therapy. The MDA5 DM phenotype in this Indian cohort is characterised by fever, cutaneous ulcers and arthritis. RPILD was not uncommon and predicted mortality.