Idiopathic pulmonary fibrosis: current classification and treatment

Idiopathic pulmonary fibrosis (IPF) is a form of the idiopathic interstitial pneumonia (IIP). It is characterized by a permanently progressive course with a high mortality. Unlike the majority of IIPs, immunosuppressive therapy has no impact on the rate of progression of IPF. In the past decade, the effectiveness of two antifibrotic drugs, that is, pirfenidone and nintedanib, was established for treatment of IPF. Therefore, timely diagnosis and earlier treatment may improve prognosis in patients with IPF. A diagnostic algorithm has been developed that requires a multidisciplinary approach with the analysis oгияf clinical, laboratory, and instrumental data, primarily lung HRCT-pattern. In case of uncertain HRCT-data, a less invasive method of transbronchial lung cryobiopsy can be used. Its diagnostic performance is comparable to that of the surgical lung biopsy. The search for biological and genetic markers of ILF is ongoing.

Key words

Idepathic pulmonary fibrosis (IPF), usual interstitial pneumonia (UIP), high resolution computed tomogrhy (HRCT), genetiс polymorhism.