• Espiridión Ramos-Martinez, Eric A Rodríguez-Vega, Pedro A Rivera-Matias, Ramcés Falfán-Valencia, Gloria Pérez-Rubio, Mayra Mejia, Montserrat I González-Pérez, Ivette Buendia-Roldán, Heidegger N Mateos-Toledo, and Jorge Rojas Serrano.
• Experimental Medicine Research Unit, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico.
• Med Clin (Barc). 2023 Dec 22; 161 (12): 515522515-522.

IntroductionThe 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.MethodsThis 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.ResultsFifty-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.ConclusionDermatological 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.Copyright © 2023 Elsevier España, S.L.U. All rights reserved.

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