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

Autor:	Ramos-Martinez E; Experimental Medicine Research Unit, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico., Rodríguez-Vega EA; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Rivera-Matias PA; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico; Becario de la Dirección General de Calidad y Educación en Salud, Secretaría de Salud, Mexico., Falfán-Valencia R; HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Pérez-Rubio G; HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Mejia M; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., González-Pérez MI; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Buendia-Roldán I; Research Direction, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Mateos-Toledo HN; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico., Serrano JR; Interstitial Lung Disease and Rheumatology Unit, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico; Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico. Electronic address: jrojas@iner.gob.mx.
Jazyk:	English; Spanish; Castilian
Zdroj:	Medicina clinica [Med Clin (Barc)] 2023 Dec 22; Vol. 161 (12), pp. 515-522. Date of Electronic Publication: 2023 Aug 09.
DOI:	10.1016/j.medcli.2023.07.013
Abstrakt:	Introduction: 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. Methods: 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. Results: 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. Conclusion: 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. (Copyright © 2023 Elsevier España, S.L.U. All rights reserved.)
Databáze:	MEDLINE
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