metricas
covid
Buscar en
Medicina Clínica (English Edition)
Toda la web
Inicio Medicina Clínica (English Edition) Clinical risk factors in patients with interstitial lung disease associated with...
Journal Information
Vol. 161. Issue 12.
Pages 515-522 (December 2023)
Share
Share
Download PDF
More article options
Vol. 161. Issue 12.
Pages 515-522 (December 2023)
Original article
Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies
Factores de riesgo clínicos en pacientes con enfermedad pulmonar intersticial con anticuerpos anti-MDA5
Espiridión Ramos-Martineza, Eric A. Rodríguez-Vegab, Pedro A. Rivera-Matiasb,c, Ramcés Falfán-Valenciad, Gloria Pérez-Rubiod, Mayra Mejiab, Montserrat I. González-Pérezb, Ivette Buendia-Roldáne, Heidegger N. Mateos-Toledob, Jorge Rojas Serranob,f,
Corresponding author
jrojas@iner.gob.mx

Corresponding author.
a Experimental Medicine Research Unit, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico
b 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
c Becario de la Dirección General de Calidad y Educación en Salud, Secretaría de Salud, Mexico
d HLA Laboratory, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico
e Research Direction, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Tlalpan, Sección XVI, 14080 Ciudad de México, Mexico
f Programa de Maestría y Doctorado en Ciencias Médicas, Facultad de Medicina, Universidad Nacional Autónoma de México, Ciudad de México. Mexico
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Figures (3)
Show moreShow less
Tables (3)
Table 1. Description of the patients included in the cohort.
Table 2. Comparison between deceased and surviving patients.
Table 3. Comparison of patients with dermatological signs associated with anti-MDA5 with those without dermatological signs.
Show moreShow less
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:
Interstitial lung disease
Anti-MDA5
Nonspecific interstitial pneumonia
Inflammatory myopathies
Resumen
Introducción

La enfermedad autoinmune asociada a los anticuerpos anti-MDA5 es una entidad poco estudiada. Los objetivos de este estudio son describir una cohorte de sujetos con enfermedad pulmonar intersticial (EPI) positivos al anticuerpo anti-MDA5 e identificar los factores clínicos de riesgo asociados con la supervivencia.

Métodos

Estudio de cohorte de un solo centro de pacientes con EPI y positivos al anticuerpo anti-MDA5. Se registraron las características clínicas basales y se realizó un análisis de supervivencia para identificar los factores de riesgo asociados con la supervivencia.

Resultados

Se incluyeron 53 pacientes con EPI y positivos a anti-MDA5; 12 pacientes fallecieron por una enfermedad intersticial rápidamente progresiva (EPI-RP). Los signos dermatológicos asociados a anti-MDA5 (pápulas de Gottron, signo de Gottron, pápulas palmares, signo de la V del escote, eritema facial de dermatomiositis y úlceras cutáneas) se asociaron fuertemente con la EPI-RP (HR: 3,7, IC 95%: 1,02-13,35). Los pacientes con manifestaciones dermatológicas eran más jóvenes, tenían mayores títulos de anticuerpos anti-MDA5, tenían mayor frecuencia de patrones inflamatorios en la tomografía de tórax de alta resolución y menor extensión de la fibrosis en la TCAR.

Conclusión

Las manifestaciones dermatológicas en los pacientes con EPI positivos a anticuerpos anti-MDA5 están asociados a EPI-RP y a desenlaces fatales al corto plazo. Los signos dermatológicos pueden identificar un subgrupo de pacientes positivos a anti-MDA5 con mayor riesgo de EPI-RP.

Palabras clave:
Enfermedad pulmonar intersticial
Anti-MDA5
Neumonía intersticial no específica
Miopatías inflamatorias

Article

These are the options to access the full texts of the publication Medicina Clínica (English Edition)
Subscriber
Subscriber

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Subscribe
Subscribe to

Medicina Clínica (English Edition)

Purchase
Purchase article

Purchasing article the PDF version will be downloaded

Price 19.34 €

Purchase now
Contact
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
E-mail
Article options
es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos