Russian Registry of Idiopathic Pulmonary Fibrosis: Clinical Features, Treatment Management, and Outcomes

SIMPLE SUMMARY: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease of unknown etiology characterized by the rapid development of respiratory failure and a poor outcome. No effective treatment is available worldwide to stop the progression of this disease and to recover the fibrotic lesions in the lungs. Registries of such patients help to better understand this disease, to make a diagnosis as early as possible, and to find the optimal treatment. We present the results of the first experience in our country of integrating the findings of patients with IPF in a registry. This experience highlighted the difficulties in making the diagnosis of IPF and choosing treatment options. We also compared the Russian population of patients with IPF with registries of similar patients in other countries. This allows further perspectives in management of this disease to be defined. ABSTRACT: A registry of patients with idiopathic pulmonary fibrosis (IPF) was founded in Russia in 2016. The aim of this study was to analyze the demographic, clinical, functional, radiological, and morphological data of the patients included in this registry. Methods. This was a prospective multicenter, observational, non-interventional study. Patients’ risk factors, demographics, clinical data, results of high-resolution computed tomography (HRCT) of the chest and pulmonary function testing, and lung tissue biopsy findings were analyzed. We also analyzed the exercise tolerance (6-min walking test) of patients, serological markers of systemic connective tissue diseases, treatment, clinical course, and outcomes of the disease. Multidisciplinary discussion (MDD) was used as needed. Results. One thousand three hundred and fifty-three patients were included in the registry from 2016 to 2020. The mean age was 64.4 ± 10.7 years, most patients were active smokers or ex-smokers. Antifibrotic therapy was administered to 90 of 948 patients (9.5%). Since starting the registry in 2016, the incidences of IPF have increased and the time period from manifestation of the disease to making the diagnosis has shortened, the number of patients on antifibrotic therapy has increased and the number of patients taking systemic steroids decreased. Conclusion. The registry of patients with IPF was helpful to improve IPF diagnosis and to implement antifibrotic agents in clinical practice. Further analysis of the clinical course and prognostic markers of IPF in the Russian population is needed. An analysis of the long-term efficacy of antifibrotic therapy in this population is also important.