Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course — Schafer 2020

Among the interstitial lung disorders (ILDs), idiopathic pulmonary fibrosis (IPF) stands out due to its unrelenting progression and unique medical management. The prognosis is poorer than many carcinomas, with a median survival of only 2-3 years after diagnosis. IPF requires a particular medical therapy since, unlike other ILDs, it does not respond to anti-inflammatory treatment with corticosteroids. This can increase survival even while the condition cannot be cured. The only treatment for increasing lung fibrosis is lung transplantation. Individual and impossible to predict, the clinical course. Acute exacerbations hasten the clinical progression and increase death. Recent studies have concentrated on the underlying pathomechanisms of IPF, with its intricate immunological and inflammatory processes and external effects. Much of its natural history is attributed to societal and environmental factors. The risks of smoking, using pneumotoxic drugs, and breathing in dusts are well documented. Hereditary and genetic variables are also very important. This concise review focuses on IPF's unique characteristics within the ILD family, particularly in respect to its underlying mechanisms and clinical development.

Schäfer SC, Funke-Chambour M, Berezowska S. Idiopathische Lungenfibrose – Epidemiologie, Ursachen und klinischer Verlauf [Idiopathic pulmonary fibrosis-epidemiology, causes, and clinical course]. Pathologe. 2020 Feb;41(1):46-51. German. doi: 10.1007/s00292-019-00747-x. PMID: 31993696.