Therapeutic options in refractory anti-MDA5 dermatomyositis triggered by SARS-CoV-2 infection

Abstract

Anti-MDA-5 dermatomyositis (DM) is a rare disease subtype characterized by rapidly progressive interstitial lung disease alongside skin, vascular and visceral involvement. The severity of the pulmonary disease is corelated with a weaker prognosis. A 31-year-old female patient was admitted for hand arthritis, upper girdle pain and multiple erythematous papular facial lesions on the face, digital ulcerations and Raynaud’s. The symptoms started immediately after the onset of the SARSCoV-2 infection. Positive anti-MDA5 and anti-centromere antibodies were identified, thus amyopathic DM - CREST syndrome overlap diagnosis was placed. Due to pulmonary involvement, corticosteroids, immunomodulatory drugs (mycophenolate mofetil, hydroxychloroquine, cyclophosphamide) were initiated, with no clinical benefits. Lack of response led to initiation of rituximab, oral tacrolimus and nintedanib with no apparent progression of the disease. Refractory cases require escalation of the standard therapeutic approach. SARS-CoV-2 infection can represent a trigger of autoimmunity in DM which needs to be further assessed.