Nashatyreva Maria S, Trofimenko Irina N, Chernyak Boris A, Avdeev Sergey N
Irkutsk State Medical Academy of Postgraduate Education-Branch Campus of the Federal State Budgetary Educational Institution of Further Professional Education "Russian Medical Academy of Continuing Professional Education", Healthcare Ministry of the Russian Federation, mkr Yubilejnyy 100, Irkutsk 664079, Russia.
Federal State Autonomous Educational Institution of Higher Education I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, 8 Trubetskaya Str, Build. 2, Moscow 119991, Russia.
Life (Basel). 2023 Jan 11;13(1):212. doi: 10.3390/life13010212.
Interstitial lung diseases (ILD) are part of a large heterogeneous group of diseases that differ in many ways (in their cause, clinical presentation, and response to therapy, etc.), but there are similar pathophysiological mechanisms involved in the development of the inflammation and/or fibrosis of the lungs. Currently, several criteria for pulmonary fibrosis (PF) and progressive pulmonary fibrosis (PPF) are proposed, and the information on the prevalence and characteristics of these conditions is limited. The aim of this study was to evaluate the spectrum of PF and PPF according to the registry of patients with ILD in eastern Siberia. Materials and methods: The study included patients with ILD from all of the medical institutions in the Irkutsk region (eastern Siberia). Each case of ILD (n = 270) was reviewed by a multidisciplinary discussion panel. The ILD patient registry included information on the clinical findings, history, pulmonary function tests, high-resolution computed tomography (HRCT), and histological findings. The follow-up period for the patients varied from 1 to 5 years. Results: Pulmonary fibrosis was detected by HRCT in 104 patients with ILD (38.5%). PF was present in 100% of the patients with IPF and SS-ILD, in 90.9% of the patients with CHP, in 71.4% of the patients with NSIP, and in 60% of the patients with RA-ILD. Sixty-two patients met the criteria for PPF (23.0% of the entire ILD cohort and 59.6% of the patients with PF). PPF occurred most often in the patients with IPF, CHP, IPAF, and SSc-ILD: 100%, 72.7%, 40%, and 38.5% of them, respectively. The variables associated with fibrosis progression included Velcro crackles (OR 18.3, p < 0.001) and late diagnosis (OR 4.1, p < 0.001). Conclusion: Pulmonary fibrosis and progressive pulmonary fibrosis are common in patients with ILD. The high mortality rate of PPF dictates the need for the active, early detection of a progressive fibrosing course of a wide range of ILD and suggests that further studies assessing the effectiveness of the interventions might be warranted.
间质性肺疾病（ILD）是一大类异质性疾病的一部分，这些疾病在许多方面（病因、临床表现和对治疗的反应等）存在差异，但在肺部炎症和/或纤维化的发展过程中涉及相似的病理生理机制。目前，已提出了几种肺纤维化（PF）和进行性肺纤维化（PPF）的标准，而关于这些病症的患病率和特征的信息有限。本研究的目的是根据东西伯利亚地区ILD患者登记处的数据评估PF和PPF的范围。材料与方法：该研究纳入了来自伊尔库茨克地区（东西伯利亚）所有医疗机构的ILD患者。每例ILD患者（n = 270）均由多学科讨论小组进行评估。ILD患者登记处包含了临床检查结果、病史、肺功能测试、高分辨率计算机断层扫描（HRCT）和组织学检查结果等信息。患者的随访期为1至5年。结果：104例ILD患者（38.5%）通过HRCT检测出肺纤维化。IPF和SS-ILD患者中100%存在PF，CHP患者中90.9%存在PF，NSIP患者中71.4%存在PF，RA-ILD患者中60%存在PF。62例患者符合PPF标准（占整个ILD队列的23.0%，占PF患者的59.6%）。PPF最常发生于IPF、CHP、IPAF和SSc-ILD患者中，分别占这些患者群体的10%、72.7%、40%和38.5%。与纤维化进展相关的变量包括Velcro啰音（比值比18.3，p < 0.001）和诊断延迟（比值比4.1，p < 0.001）。结论：肺纤维化和进行性肺纤维化在ILD患者中很常见。PPF的高死亡率表明需要积极、早期地检测广泛的ILD的进行性纤维化病程，并提示可能有必要进一步开展评估干预措施有效性的研究。
