Federal clinical guidelines on diagnosis and treatment of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrotic interstitial pneumonia with a progressive course and poor prognosis. The prevalence of IPF in the Russian Federation is about 8 – 12 cases per 100,000 population. The basic treatment of IPF is antifibrotic agents. Methodology . The target audience of these clinical recommendations are therapists, general practitioners, pulmonologists, pathologists, radiologists, and medical rehabilitation doctors. Each thesis-recommendation about diagnostic and therapeutic procedures has been scored according to the scale of classes of recommendations from 1 to 5 and A, B, C scale of the levels of evidence. Clinical recommendations also contain comments and explanations to the theses, algorithms for the diagnosis and treatment of idiopathic pulmonary fibrosis, and reference materials. Conclusion . The presented clinical guidelines cover current information about the etiology and pathogenesis, clinical manifestations, diagnosis, treatment, and prevention of idiopathic pulmonary fibrosis. These guidelines were approved by the Scientific and Practical Council of the Ministry of Health of the Russian Federation in 2021.