Idiopathic pulmonary fibrosis (IPF) is a severe lung disease, with death occurring within 2-5 years after its onset. IPF affects people in the second half of life. Its causes are unknown. Before 1999, IPF was out from the group of idiopathic interstitial lung diseases as a separate nosological entity. Practitioners very often (80%) make diagnostic errors in IPF and prescribe antibiotics, anti-inflammatory drugs, which worsen the course of this disease. The distinctive feature of the pathogenesis of IPF is the absence of inflammation, which is clinically manifested by the inefficacy of glucocorticosteroids and other anti-inflammatory drugs. Pharmacological agents for the treatment of IPF have been designed since 2000. One of them has been registered and permitted for use in the Russian Federation. This paper is a review of an update on the problem of IPF, which should facilitate the appropriate orientation of physicians in diagnosing and treating this severe disease.
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