Reclassification of Fibrotic Interstitial Lung Diseases through Environmental and Genetic Studies

Pulmonary fibrosis is characterized by irreversible damage to lung function and represents the end stage of several progressive interstitial lung diseases (ILDs). This heterogeneous group of pathologies results from complex interactions between genetic factors and environmental factors, which modulate the immune response and, consequently, trigger the fibrotic cascade in susceptible individuals. Current treatment of ILD focuses on accurately identifying its etiology. However, while idiopathic pulmonary fibrosis (IPF) is the classic model of fibrosing disease, other ILDs can also progress to a self-sustaining fibrotic process, even with conventional immunomodulatory therapy.

FIREGENS builds on the solid foundation established by the extensive cohort of ILD patients developed within the FIBRALUNG project, as well as the ongoing efforts of biobanks to systematically collect and store a growing number of biological samples. This new initiative aims to conduct comprehensive research on environmental factors and host characteristics, focusing on three distinct diagnostic groups: hypersensitivity pneumonitis (HP), IPF, and unclassifiable ILD. The primary objective is to identify common risk factors associated with the development and progression of pulmonary fibrosis, regardless of the primary ILD diagnosis.

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PI: Hélder Novais-Bastos

SPONSOR
Faculdade de Medicina da Universidade do Porto
FUNDING