Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies

• DOI: 10.1016/j.medcli.2023.07.013
• Publication Date: 2023-08-09T19:59:54Z
• Authors: Espiridión Ramos-...; Eric A. Rodríguez...; Pedro A. Rivera-M...; Ramcés Falfán-Val...; Gloria Pérez-Rubio; Mayra Mejia; Montserrat I. Gon...; Ivette Buendia-Ro...; Heidegger N. Mate...; Jorge Rojas Serrano
• Keywords: Cohort Studies; Interferon-Induced Helicase, IFIH1; Risk Factors; Humans; Lung Diseases, Interstitial; 3. Good health; Autoantibodies; Retrospective Studies

The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival.This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival.Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02-13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT.Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD.