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. 2023 Feb 3;13(2):435.
doi: 10.3390/life13020435.

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

Svetlana Chikina  1 , Alexander Cherniak  2 , Zamira Merzhoeva  1 , Igor Tyurin  3 , Natalia Trushenko  1   2 , Anna Proshkina  1 , Kirill Ataman  1 , Sergey Avdeev  1   2
Affiliations

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

Svetlana Chikina et al. Life (Basel). .

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.

Keywords: antifibrotic agents; idiopathic pulmonary fibrosis; multidisciplinary discussion; registry.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Time between onset of first symptoms and diagnosis of IPF (years). (Data are presented as absolute number of patients).
Figure 2
Figure 2
Distribution of comorbidities in patients with IPF at baseline. (Data are presented as percentage of all patients).
Figure 3
Figure 3
Distribution of symptoms and signs of IPF patients. Data are presented as percentage of all patients with reported symptoms or signs.
Figure 4
Figure 4
Distribution of FVC data at baseline of IPF patients. Data are presented as percentage of all patients.
Figure 5
Figure 5
Distribution of DLCO data at baseline of IPF patients. Data are presented as percentage of all patients.
Figure 6
Figure 6
Changes in therapies of patients with IPF while registry was running. NAC, N-acetylcysteine; CFA, cyclophosphamide; AZA, azathioprine; PIRF, pirfenidone; NINT, nintedanib; MMF, mycophenolate mofetil; PPI, proton pump inhibitors. * p < 0.01.
See this image and copyright information in PMC

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