Abstract
Interstitial lung diseases (ILDs) is a large and heterogeneous group of disorders with a variable degree of lung inflammation and lung fibrosis. In some ILDs, we can observe a progressive-fibrosing phenotype-PF-ILD (e.g., idiopathic pulmonary fibrosis, fibrotic phenotype of hypersensitivity pneumonitis, familial lung fibrosis, etc.). Lung fibrosis is characterized by overgrowth, stiffening, and scarring of tissues due to excess deposition of extracellular matrix. In some patients suffering from PF-ILD, progression and fatal outcomes occur despite treatment. Therefore, there is a great need for the development of lung fibrosis models that will help to understand and recapitulate the etiopathogenesis of the disease and may thus serve as tools for unraveling its underlying profibrotic mechanisms and potential therapeutic targets. In this review, we summarize ILD etiopathogenesis, current and novel therapeutic options, and discuss in vivo, ex vivo, and in vitro near-to-native lung fibrosis models, which help to elucidate specific processes within ILD pathophysiology.