Journal Information
Vol. 19. Issue 4.
Pages 211-214 (April 2023)
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Vol. 19. Issue 4.
Pages 211-214 (April 2023)
Brief Report
Progressive pulmonary fibrosis in systemic autoimmune diseases. A real life study
Fibrosis pulmonar en enfermedades autoinmunes sistémicas. Un estudio en vida real
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Diego Durán Barataa,
Corresponding author
iago_794@hotmail.com

Corresponding author.
, Ana Jaureguizar Oriola, Jesús Loarce Martosb, Jose Luis Morell Hitab, Carlos de la Puente Bujidosb, Juan Rigual Bobilloa
a Servicio de Neumología, Hospital Universitario Ramón y Cajal, Madrid, Spain
b Servicio de Reumatología, Hospital Universitario Ramón y Cajal, Madrid, Spain
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Abstract
Introduction

Interstitial lung diseases associated with systemic autoimmune diseases (ILD-SAD) can progress to a fibrotic form that can benefit from antifibrotic treatment. The aim of the study is to describe a cohort of patients with ILD-SAD who manifest progressive pulmonary fibrosis treated with antifibrotics.

Methods

Single-centre retrospective observational study from a tertiary care hospital on a cohort of patients with ILD-SAD with progressive pulmonary fibrosis evaluated in a joint pulmonology and rheumatology clinic that initiated treatment with antifibrotic drugs between 01/01/2019 and 01/12/2021. Clinical characteristics were analysed. The evolution of pulmonary function test and adverse effects during treatment were described.

Results

18 patients were included. The mean age was 66.7 ± 12.7 years, with a higher frequency of females (66.7%). Systemic sclerosis (SS) was the most frequent systemic autoimmune disease (36.8%). The majority of patients were receiving systemic glucocorticoid treatment (88.9%), 72.2% of patients were receiving treatment with disease-modifying drugs, the most frequent being mycophenolate mofetil (38.9%), and 22.2% with rituximab. Functional stability was observed after the start of antifibrotic treatment. Two patients died during follow-up, one due to progression of ILD.

Conclusion

Our study suggests a beneficial effect of antifibrotic treatment added to immunomodulatory treatment in patients with fibrotic ILD-SAD in real life. In our cohort, patients with ILD-SAD with progressive fibrosing involvement show functional stability after starting antifibrotic treatment. Treatment tolerance was relatively good with a side effect profile similar to that described in the medical literature.

Keywords:
Interstitial lung disease
Systemic autoimmune disease
Progressive fibrosing ILD
Antifibrotics
Resumen
Introducción

Las enfermedades pulmonares intersticiales difusas asociadas a enfermedades autoinmunes sistémicas (EPID-EAS) pueden presentar una progresión fibrótica. El objetivo principal del estudio es describir una serie de casos de pacientes con EPID-EAS que cursan con fibrosis pulmonar progresiva e inician tratamiento con fármacos antifibróticos.

Métodos

Estudio observacional retrospectivo unicéntrico de un hospital de tercer nivel sobre una serie de casos de pacientes con EPID-EAS con fibrosis pulmonar progresiva valorados en una consulta conjunta de neumología y reumatología, que iniciaron tratamiento con fármacos antifibróticos entre 01/01/2019 y 01/12/2021. Se analizaron las características epidemiológicas, clínicas, funcionales, radiológicas y terapéuticas al inicio del tratamiento, y la evolución funcional durante el tratamiento, así como los efectos adversos.

Resultados

Se incluyeron 18 pacientes. La edad media observada fue de 66.7 ± 12.7 años con mayor frecuencia de sexo femenino (66.7%), siendo la esclerosis sistémica la enfermedad autoinmune sistémica más frecuente (36.8%). La mayoría de los pacientes se encontraban con tratamiento con glucocorticoides sistémicos (88.9%), un 72.2% de pacientes con fármacos modificadores de la enfermedad, siendo el más frecuente el micofenolato mofetilo (38.9%), y un 22.2% con rituximab. Se observó una estabilidad funcional tras el inicio del tratamiento con antifibrótico. Fallecieron dos pacientes durante el seguimiento, uno como consecuencia de la progresión de la enfermedad intersticial pulmonar.

Conclusión

Nuestro estudio sugiere un efecto beneficioso del tratamiento antifibrótico añadido al tratamiento inmunomodulador en pacientes con EPID-EAS fibrótica en vida real. En nuestra serie de casos, los pacientes con EPID-EAS con afectación fibrosante progresiva muestran una estabilidad funcional tras el inicio del tratamiento antifibrótico. La tolerancia al tratamiento fue relativamente buena, con un perfil de efectos secundarios similar al descrito en la literatura médica.

Palabras clave:
Enfermedad intersticial difusa
Enfermedad autoinmune sistémica
EPID fibrosante progresiva
Antifibróticos

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