A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.

• Klíčová slova
• Connective tissue diseases, Mortality, Pulmonary fibrosis, Rheumatic diseases, Systemic sclerosis, Vital capacity,
• MeSH
• hypersenzitivní pneumonitida diagnostické zobrazování   epidemiologie   MeSH
• idiopatická plicní fibróza diagnostické zobrazování   epidemiologie   MeSH
• intersticiální plicní nemoci diagnostické zobrazování   epidemiologie   MeSH
• lidé MeSH
• mortalita trendy   MeSH
• progrese nemoci * MeSH
• rizikové faktory MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH