Clinical characteristics and outcomes in patients with Anti-MDA5 positive interstitial lung disease: A case series from a lung transplant center
Abstract
Background Anti-Melanoma Differentiation-5 (MDA5)-positive dermatomyositis (DM) is a rare autoimmune disorder frequently complicated by rapidly progressive interstitial lung disease (RP-ILD), often with poor outcomes. Data from European tertiary care and transplant centers remain limited. Objective To describe the clinical presentation, treatment course, and outcomes of five patients with anti-MDA5+DM-associated ILD at a lung transplant center. Methods We retrospectively reviewed five patients with confirmed anti-MDA5 seropositivity and interstitial lung involvement between 2023 and 2025. Demographic data, clinical features, lung function, imaging, antibody levels, immunosuppressive treatment, and outcomes were analyzed. Results Of the five patients 3 were female and two were male with a median age of 51 years (range 22–69). Clinical severity varied from mild cutaneous and pulmonary disease to fulminant RP-ILD. One patient required extracorporeal membrane oxygenation (ECMO) and eventually underwent lung transplantation. Treatment strategies included high-dose corticosteroids, rituximab, mycophenolate mofetil, tacrolimus, and intravenous immunoglobulins (IVIG). Patients with early initiation of rituximab or IVIG and declining anti-MDA5 antibody titers generally showed pulmonary stabilization. Serial lung function tests correlated with clinical improvement or deterioration. Cutaneous flare-ups persisted in some patients despite pulmonary stability. Conclusion This case series…
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