Antifibrotic agents in progressive pulmonary fibrosis and non-progressive pulmonary fibrosis of fibrotic hypersensitivity pneumonitis

IF 2.4 Q2 RESPIRATORY SYSTEM

Respiratory investigation Pub Date : 2025-06-20 DOI:10.1016/j.resinv.2025.06.004

Taichi Kaneko , Ryo Okuda , Tamiko Takemura , Tae Iwasawa , Sanshiro Haga , Yuriko Takeda , Eri Hagiwara , Takashi Ogura

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Abstract

Background

In patients with fibrotic hypersensitivity pneumonitis (HP), the effect of antifibrotic agents in those with progressive pulmonary fibrosis (PPF) and non-PPF remain unclear. This study aims to evaluate the effects of antifibrotic agents in patients with fibrotic HP by comparing outcomes between the PPF and non-PPF groups.

Methods

This single-center retrospective study included patients diagnosed with pathologically confirmed fibrotic HP who received antifibrotic agents. Patients were classified as PPF or non-PPF based on disease progression in the year before antifibrotic treatment.

Results

In this study, 63 were classified as PPF and 33 as non-PPF. Overall survival differed significantly between the groups (hazard ratio [HR] = 3.28, 95 % confidence interval [CI]: 1.48–7.28, p = 0.002). The incidence of acute exacerbation was also higher in the PPF group (HR = 2.45, 95 % CI: 1.05–5.75, p = 0.033). In the PPF group, the annual forced vital capacity (FVC) decline improved from −11.5 % before treatment to −4.2 % in the first year after treatment (p < 0.001). In the non-PPF group, the annual FVC change before and after treatment was not significantly different (1.6 % vs. −1.7 %, p = 0.065). Serum Krebs von den Lungen-6 (KL-6) levels decreased in the PPF group after 12 months of treatment (24.9 % vs. −2.7 %, p = 0.002), while no significant change was observed in the non-PPF group (8.6 % vs. 4.3 %, p = 0.800).

Conclusions

In fibrotic HP, antifibrotic agents seemed to contribute to a reduction in FVC decline and lower KL-6 levels in patients with PPF.

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抗纤维化药物在进行性肺纤维化和非进行性肺纤维化纤维化过敏性肺炎中的应用

在纤维化超敏性肺炎（HP）患者中，抗纤维化药物在进行性肺纤维化（PPF）和非PPF患者中的作用尚不清楚。本研究旨在通过比较PPF组和非PPF组的结果来评估抗纤维化药物对纤维化HP患者的影响。方法本研究为单中心回顾性研究，纳入病理确诊的纤维化性HP患者并接受抗纤维化药物治疗。根据抗纤维化治疗前一年的疾病进展将患者分为PPF或非PPF。结果本组63例为PPF， 33例为非PPF。两组总生存率差异有统计学意义（风险比[HR] = 3.28, 95%可信区间[CI]: 1.48 ~ 7.28, p = 0.002）。PPF组急性加重发生率也较高（HR = 2.45, 95% CI: 1.05 ~ 5.75, p = 0.033）。在PPF组，年强迫肺活量（FVC）下降从治疗前的- 11.5%改善到治疗后第一年的- 4.2% (p <；0.001)。非ppf组治疗前后FVC年变化无显著性差异（1.6% vs. - 1.7%, p = 0.065）。治疗12个月后，PPF组血清Krebs von den Lungen-6 （KL-6）水平下降（24.9%对- 2.7%,p = 0.002），而非PPF组无显著变化（8.6%对4.3%,p = 0.800）。结论在纤维化HP中，抗纤维化药物似乎有助于降低PPF患者FVC下降和KL-6水平。

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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