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Vol. 31. Issue S1.
Interstitial Lung Disease Associated with Autoimmune Diseases
Pages S132-S138 (April 2024)
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Vol. 31. Issue S1.
Interstitial Lung Disease Associated with Autoimmune Diseases
Pages S132-S138 (April 2024)
Special article
Genetics of autoimmune-associated interstitial lung diseases: A focus on rheumatoid arthritis
Genética de las enfermedades pulmonares intersticiales autoinmunes asociadas: un enfoque sobre la artritis reumatoide
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Philippe Dieudéa,b
a Université de Paris, INSERM UMR 1152, Paris, France
b Rheumatology Service, Hôpital Bichat-Claude Bernard, AP-HP, Paris, France
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Special issue
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Vol. 31. Issue S1

Interstitial Lung Disease Associated with Autoimmune Diseases

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Abstract

Recent advances in deciphering the genetic architecture of RA-ILD support the hypothesis of RA-ILD as a complex disease with a heterogeneous phenotype encompassing at least the usual interstitial pneumonia (UIP) and non-UIP high-resolution CT patterns. The results of genetic studies support the hypothesis of a common genetic background between idiopathic pulmonary fibrosis (IPF) and RA-ILD, and more specifically RA-UIP, a subset of the disease associated with a poor prognosis. Overall, these findings suggest the existence of shared pathogenic pathways between IPF and RA-ILD providing new opportunities for future intervention in RA-ILD, particularly with drugs that have been shown to be active in IPF.

Keywords:
Rheumatoid arthritis
Interstitial lung disease
MUC5B
Resumen

Los recientes avances en el desciframiento de la arquitectura genética de la artritis reumatoide asociada a enfermedad pulmonar intersticial (EPI-AR) apoyan la hipótesis de que la EPI-AR es una enfermedad compleja, con un fenotipo heterogéneo, que abarca al menos los patrones de neumonía intersticial habitual (NIU) y de TC de alta resolución no NIU. Los resultados de los estudios genéticos apoyan la hipótesis de un trasfondo genético común entre la fibrosis pulmonar idiopática (FPI) y la EPI-AR, y más concretamente la NIU-AR, un subconjunto de la enfermedad asociado a un mal pronóstico. En conjunto, estos hallazgos sugieren la existencia de vías patogénicas compartidas entre la FPI y la EPI-AR que proporcionan nuevas oportunidades para futuras intervenciones en la EPI-AR, particularmente con fármacos que han demostrado ser activos en la FPI.

Palabras clave:
Artritis reumatoide
Enfermedad pulmonar intersticial
MUC5B

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