Chikina Svetlana, Cherniak Alexander, Merzhoeva Zamira, Tyurin Igor, Trushenko Natalia, Proshkina Anna, Ataman Kirill, Avdeev Sergey
Department of Pulmonology, Sechenov First Moscow State Medical University (Sechenov University), 8, Build.2, Trubetskaya Str., Moscow 119991, Russia.
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia, 28, Orehovyi Bul., Moscow 115682, Russia.
Life (Basel). 2023 Feb 3;13(2):435. doi: 10.3390/life13020435.
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.
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.
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.
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.
2016年在俄罗斯建立了特发性肺纤维化（IPF）患者登记处。本研究的目的是分析该登记处纳入患者的人口统计学、临床、功能、放射学和形态学数据。
这是一项前瞻性多中心观察性非干预研究。分析了患者的危险因素、人口统计学、临床数据、胸部高分辨率计算机断层扫描（HRCT）结果、肺功能测试以及肺组织活检结果。我们还分析了患者的运动耐量（6分钟步行试验）、系统性结缔组织病的血清学标志物、治疗、临床病程和疾病结局。必要时采用多学科讨论（MDD）。
2016年至2020年登记处共纳入1353例患者。平均年龄为64.4±10.7岁，大多数患者为现吸烟者或既往吸烟者。948例患者中有90例（9.5%）接受了抗纤维化治疗。自2016年开始登记以来，IPF的发病率有所上升，从疾病表现到确诊的时间缩短，接受抗纤维化治疗的患者数量增加，服用全身性类固醇的患者数量减少。
IPF患者登记处有助于改善IPF诊断并在临床实践中应用抗纤维化药物。需要进一步分析俄罗斯人群中IPF的临床病程和预后标志物。分析该人群中抗纤维化治疗的长期疗效也很重要。
