Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies
Med Clin (Barc). 2023 Dec 22;161(12):515-522.
doi: 10.1016/j.medcli.2023.07.013.
Epub 2023 Aug 9.
[Article in
English,
Spanish]
Affiliations
- 1 Experimental Medicine Research Unit, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico.
- 2 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.
- 3 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.
- 4 HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico.
- 5 Research Direction, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico.
- 6 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.
Abstract
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.
Keywords:
Anti-MDA5; Enfermedad pulmonar intersticial; Inflammatory myopathies; Interstitial lung disease; Miopatías inflamatorias; Neumonía intersticial no específica; Nonspecific interstitial pneumonia.
Copyright © 2023 Elsevier España, S.L.U. All rights reserved.
MeSH terms
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Autoantibodies*
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Cohort Studies
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Humans
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Interferon-Induced Helicase, IFIH1
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Lung Diseases, Interstitial* / complications
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Lung Diseases, Interstitial* / etiology
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Retrospective Studies
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Risk Factors
Substances
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Autoantibodies
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Interferon-Induced Helicase, IFIH1