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IF 2.4 Q2 RESPIRATORY SYSTEM

Respiratory investigation Pub Date : 2025-01-31 DOI:10.1016/j.resinv.2025.01.007

Masato Kono , Noriyuki Enomoto , Yusuke Inoue , Hideki Yasui , Masato Karayama , Yuzo Suzuki , Hironao Hozumi , Kazuki Furuhashi , Mikio Toyoshima , Shiro Imokawa , Masato Fujii , Taisuke Akamatsu , Naoki Koshimizu , Koshi Yokomura , Hiroyuki Matsuda , Yusuke Kaida , Yutaro Nakamura , Masahiro Shirai , Masafumi Masuda , Tomoyuki Fujisawa , Takafumi Suda

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摘要

背景：特发性间质性肺炎（IIPs）可能因临床、放射学或组织病理学发现不充分、非特异性或相互矛盾而无法分类，尽管进行了多学科讨论（MDD）。无法分类的肺结核（UCIIP）是一种异质性疾病，可表现为进行性肺纤维化（PPF）。本研究旨在调查 PPF 在 UCIIP 患者中的发病率和临床特征：在这项对 222 名 IIP 患者的前瞻性多中心登记进行的事后分析中，有 71 名通过 MDD 诊断的 UCIIP 患者被纳入研究。PPF是根据12个月内症状恶化、放射学和生理学进展来定义的，采用的是指南标准或INBUILD试验24个月内的标准：中位年龄为 72 岁，19.7% 的患者进行了手术肺活检。在 66 名有足够随访数据的患者中，有 30 人（45.5%）符合其中任一标准并被诊断为 PPF。与没有 PPF 的患者相比，患有 PPF 的 UCIIP 患者的血清表面活性物质蛋白-D 水平和支气管肺泡液中性粒细胞百分比明显更高，肺活量百分比和一氧化碳扩散能力百分比更低，高分辨率计算机断层扫描上的蜂窝状比例和用力时的不饱和程度更高。此外，与无 PPF 的患者相比，他们接受抗纤维化治疗和长期氧疗的次数明显较多，急性加重的发生率较高，预后较差。Cox比例危险分析显示，无论标准如何，PPF都是一个重要的不良预后因素：结论：PPF在UCIIP患者中很常见，且与预后不良有关。适当评估和处理 PPF 对 UCIIP 至关重要。

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Prevalence and clinical features of progressive pulmonary fibrosis in patients with unclassifiable idiopathic interstitial pneumonia: A post hoc analysis of prospective multicenter registry

Background

Idiopathic interstitial pneumonias (IIPs) may remain unclassifiable owing to inadequate, nonspecific, or conflicting clinical, radiological, or histopathological findings despite multidisciplinary discussion (MDD). Unclassifiable IIP (UCIIP) is a heterogeneous disease that can present with progressive pulmonary fibrosis (PPF). This study aimed to investigate the prevalence and clinical features of PPF in patients with UCIIP.

Methods

In this post hoc analysis of a prospective multicenter registry of 222 patients with IIPs, 71 with UCIIP diagnosed using MDD were enrolled. PPF was defined based on worsening symptoms and radiological and physiological progression using the guideline criteria within 12 months or the criteria from the INBUILD trial within 24 months.

Results

The median age was 72 years, and surgical lung biopsy was performed in 19.7%. Of the 66 patients with adequate follow-up data, 30 (45.5%) met either criterion and were diagnosed with PPF. UCIIP patients with PPF had significantly higher serum surfactant protein-D level and percentage of bronchoalveolar fluid neutrophils, lower %forced vital capacity and %diffusing capacity for carbon monoxide, and a higher proportion of honeycombing on high-resolution computed tomography and desaturation on exertion than those without PPF. Additionally, they had significantly more anti-fibrotic therapy and long-term oxygen therapy, a higher incidence of acute exacerbation, and a poorer prognosis than those without PPF. Cox proportional hazards analysis revealed that PPF was a significant poor prognostic factor, regardless of the criteria.