Acute exacerbation of progressive pulmonary fibrosis: incidence and outcomes.

IF 5.8 2区医学 Q1 Medicine Respiratory Research Pub Date : 2024-11-26 DOI:10.1186/s12931-024-03048-x

Min Jee Kim, Jiyoul Yang, Jin Woo Song

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Abstract

Background: Few data are available on acute exacerbation (AE) in patients with progressive pulmonary fibrosis (PPF) besides idiopathic pulmonary fibrosis (IPF). This study aimed to investigate the AE incidence and outcomes among patients with PPF.

Methods: Clinical data of patients with PPF (n = 133) were retrospectively collected at a single center. PPF was defined based on the criteria used in the INBUILD trial. AE was defined as a worsening of dyspnea typically within 30 days with new bilateral lung infiltration and no evidence of cardiac failure or fluid overload.

Results: Among patients with PPF, the mean age was 60.6 years old, 57.1% were females, and the most common etiology was connective tissue disease-related ILDs (63%). During the follow-up (median: 38.0 months) after PPF diagnosis, 42 patients (31.6%) experienced AE. The 1-, 3-, and 5-year AE incidences were 12.5%, 30.3%, and 38.0%, respectively. Older age, rheumatoid arthritis associated ILD, fibrotic hypersensitivity pneumonitis, and lower lung diffusing capacity for carbon monoxide were AE risk factors. Patients with AE demonstrated worse survival (median survival: 30 months vs. not reached; p < 0.001) after PPF diagnosis than those without. AE was independently associated with mortality in patients with PPF (hazard ratio [HR], 2.194; 95% confidence interval [CI], 1.285-3.747; p = 0.004) in the multivariable Cox analysis, along with older age, lower lung diffusing capacity for carbon monoxide, and the usual interstitial pneumonia-like pattern on high-resolution computed tomography.

Conclusions: Our results suggest AE is not uncommon and significantly impacts on survival in patients with PPF.

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进行性肺纤维化急性加重：发生率和结果。

背景：除特发性肺纤维化（IPF）外，有关进行性肺纤维化（PPF）患者急性加重（AE）的数据很少。本研究旨在调查 PPF 患者急性加重的发生率和结果：在一个中心回顾性收集了 PPF 患者（n = 133）的临床数据。PPF的定义基于INBUILD试验中使用的标准。AE的定义是：通常在30天内呼吸困难恶化，出现新的双侧肺部浸润，且无心力衰竭或体液超负荷的证据：在 PPF 患者中，平均年龄为 60.6 岁，57.1% 为女性，最常见的病因是结缔组织病相关的 ILD（63%）。在确诊 PPF 后的随访期间（中位数：38.0 个月），42 名患者（31.6%）出现了 AE。1年、3年和5年的AE发生率分别为12.5%、30.3%和38.0%。高龄、类风湿性关节炎相关的 ILD、纤维化超敏性肺炎和较低的一氧化碳肺弥散能力是 AE 的风险因素。有 AE 的患者生存期较短（中位生存期：30 个月 vs. 未达到；P 结论：AE 并不罕见，但在肺癌患者中，AE 的发病率较高：我们的研究结果表明，AE 并非罕见，而且对 PPF 患者的生存有显著影响。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Respiratory Research RESPIRATORY SYSTEM-

CiteScore

9.70

自引率

1.70%

发文量

314

审稿时长

4-8 weeks

期刊介绍： Respiratory Research publishes high-quality clinical and basic research, review and commentary articles on all aspects of respiratory medicine and related diseases. As the leading fully open access journal in the field, Respiratory Research provides an essential resource for pulmonologists, allergists, immunologists and other physicians, researchers, healthcare workers and medical students with worldwide dissemination of articles resulting in high visibility and generating international discussion. Topics of specific interest include asthma, chronic obstructive pulmonary disease, cystic fibrosis, genetics, infectious diseases, interstitial lung diseases, lung development, lung tumors, occupational and environmental factors, pulmonary circulation, pulmonary pharmacology and therapeutics, respiratory immunology, respiratory physiology, and sleep-related respiratory problems.