Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies.
Med Clin (Barc)
; 161(12): 515-522, 2023 12 22.
Article
en En, Es
| MEDLINE
| ID: mdl-37567825
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.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Autoanticuerpos
/
Enfermedades Pulmonares Intersticiales
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prognostic_studies
/
Risk_factors_studies
Límite:
Humans
Idioma:
En
/
Es
Revista:
Med Clin (Barc)
Año:
2023
Tipo del documento:
Article