Gaëlle Schils : Fibrose pulmonaire idiopathique canine : update et perspectives

Saturday, February 28, 2026 - Public Session Canine idiopathic pulmonary fibrosis (CPF) is a progressive, chronic, and irreversible disease characterized by the deposition of extracellular matrix in the lung parenchyma. It primarily affects older West Highland White Terriers. Its etiology remains unknown, but a genetic predisposition coupled with the influence of environmental factors is strongly suspected. The median survival after diagnosis is approximately one year, and there is no specific treatment. Management therefore focuses on managing complications, particularly pulmonary arterial hypertension, as well as comorbidities and clinical symptoms. This disease shares many similarities with human idiopathic pulmonary fibrosis, notably an increased incidence of lung carcinoma development. Furthermore, affected dogs represent interesting spontaneous translational models, complementary to classical models. Among the biomarkers and therapeutic targets recently studied, fibroblast activation protein (FAP) and programmed cell death ligand 1 (PD-L1) stand out. FAP expression in areas of active fibrosis led to the development of positron emission tomography using a fluorine-18 coupled FAP inhibitor (FAPI), a valuable tool for monitoring response in clinical trials. Consistent with data available in human idiopathic pulmonary fibrosis and murine models, PD-L1 also appears to be overexpressed in canine idiopathic pulmonary fibrosis and may be involved in its pathogenesis. Finally, the recent publication of a comprehensive molecular atlas of healthy canine lung cells has provided an essential reference framework for the study of canine lung diseases.