Idiopathic pulmonary fibrosis Registry in Russia

Russian registry of patients with idiopathic pulmonary fibrosis (IPF) is established in 2016.

The aim of this study was to describe demographic, clinical, functional, radiological and pathomorphological features of IPF in Russian population of patients.

Methods. This was a prospective multicenter non-interventional observational study. We analyzed risk factors, demographic and clinical findings, CT findings, lung function tests, lung tissue biopsy, 6-minute walking test results, serological markers of connective tissue diseases, treatment, and outcomes.

Results. The Russian IPF registry has included 1,033 patients to the 11th of March, 2020. The patients’ mean age was 64.6 ± 10.8 years. The patients were males and current or former smokers more often. Multidisciplinary discussion was used in 38 patients. IPF was diagnosed in 268 (27.3%) of 980 patients. Non-IPF fibrosing interstitial lung diseases (ILDs) including chronic hypersensitivity pneumonitis, unclassifiable pulmonary fibrosis and other non-ILD pulmonary diseases were diagnosed in others. Ninety of 948 (9.5 %) patients were treated with antifibrotics. Number of diagnosed IPF cases increased, the time from IPF manifestation to making the diagnosis shortened after the registry was created.

Conclusion. Patients were treated less often with systemic corticosteroids and more often with antifibrotics during the registry existence. 

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