[Potential implication of the IgA-pIgR system in idiopathic pulmonary fibrosis]

Abstract

Idiopathic pulmonary fibrosis (IPF) is a lethal respiratory disease characterized by the excessive deposition of extracellular matrix in the alveolar zones. The bronchiolar epithelium has been implicated in the development of this disease and is capable of secreting IgA into the airway lumen thanks to its expression of the polymeric immunoglobulin receptor. Several elements indicate a dysregulation of this system, such as raised serum IgA levels in IPF patients and the pro-fibrotic effect of IgA on several key cell types. Our work aims at studying the underlying mechanisms so as to better understand the role of IgA mucosal immunity in this disease.

Keywords: Fibrose pulmonaire idiopathique; Idiopathic pulmonary fibrosis; Immunoglobulin A; Immunoglobuline A; Polymeric immunoglobulin receptor; Récepteur des immunoglobulines polymériques.