Predictors of one-year interstitial lung disease progression in patients with interstitial pneumonia with autoimmune features: a single-center, prospective study.

IF 4.7 4区医学 Q1 MEDICINE, GENERAL & INTERNAL

Polskie Archiwum Medycyny Wewnetrznej-Polish Archives of Internal Medicine Pub Date : 2025-09-08 DOI:10.20452/pamw.17115

Ewa Miądlikowska, Joanna Miłkowska-Dymanowska, Adam J Białas, Joanna S Makowska, Anna Lewandowska-Polak, Piotr Łacwik, Wojciech J Piotrowski

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引用次数: 0

Abstract

Introduction: Interstitial pneumonia with autoimmune features (IPAF) describes a rare condition characterized by interstitial lung disease (ILD) with autoimmune manifestations in the absence of defined autoimmune rheumatic diseases (AIRD). Although the classification was established in 2015, prospective data on disease progression remain limited.

Objectives: To identify predictors of ILD progression in IPAF patients using three criteria: 1) progressive pulmonary fibrosis (PPF), 2) INBUILD criteria, 3) absolute FVC decline ≥10%. A secondary aim was to compare bronchoalveolar lavage fluid (BALF) cellular profiles between IPAF and AIRD-ILD and explore their prognostic value.

Patients and methods: Between March 2021 and November 2022, 69 patients were prospectively recruited: 31 with IPAF and 38 with AIRD-ILD. Clinical, laboratory, radiological, pulmonary function and BALF data were analyzed. Univariable and multivariable logistic regression were used to assess one-year progression predictors.

Results: Among IPAF patients, 43.3% met PPF criteria, 36.7% met INBUILD and 22.2% had FVC decline ≥10%. PPF-defined progression was more frequent in IPAF than AIRD-ILD (43.3% vs. 19.35%, p = 0.04). Predictors of progression per PPF criteria included elevated peripheral blood neutrophil count (OR = 1.89, 95% CI: 1.08-3.31; p = 0.03) and usual interstitial pneumonia pattern on HRCT (OR = 43.8, 95% CI: 1.52-1260; p = 0.03). BALF lymphocyte count was not associated with progression (OR = 1.10, p = 0.07). For INBUILD criteria, predictors were peripheral blood neutrophil count (OR = 2.06, 95% CI: 1.19-3.59; p = 0.01) and honeycombing (OR = 69.1, 95% CI:2.16-2220; p = 0.02). Antinuclear antibody titer had no impact on IPAF progression.

Conclusions: Fibrotic HRCT features (UIP, honeycombing) and peripheral neutrophilia were predictors of ILD short-term progression in IPAF.

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具有自身免疫性特征的间质性肺炎患者一年间质性肺病进展的预测因素：一项单中心前瞻性研究

简介：间质性肺炎伴自身免疫性特征（IPAF）描述了一种罕见的疾病，其特征为间质性肺疾病（ILD），在没有明确的自身免疫性风湿病（AIRD）的情况下具有自身免疫性表现。尽管该分类于2015年建立，但关于疾病进展的前瞻性数据仍然有限。目的：通过三个标准确定IPAF患者ILD进展的预测因素：1)进行性肺纤维化（PPF）， 2) INBUILD标准，3)绝对FVC下降≥10%。第二个目的是比较IPAF和AIRD-ILD之间的支气管肺泡灌洗液（BALF）细胞谱，并探讨其预后价值。患者和方法：在2021年3月至2022年11月期间，前瞻性招募了69名患者：31名IPAF患者和38名AIRD-ILD患者。分析临床、实验室、放射学、肺功能和BALF数据。采用单变量和多变量logistic回归评估一年进展预测因子。结果：在IPAF患者中，43.3%的患者符合PPF标准，36.7%的患者符合INBUILD标准，22.2%的患者FVC下降≥10%。ppf定义的进展在IPAF中比AIRD-ILD更常见（43.3%比19.35%,p = 0.04）。PPF标准进展的预测因子包括外周血中性粒细胞计数升高（OR = 1.89, 95% CI: 1.08-3.31; p = 0.03）和HRCT上常见的间质性肺炎（OR = 43.8, 95% CI: 1.52-1260; p = 0.03）。BALF淋巴细胞计数与进展无关（OR = 1.10, p = 0.07）。对于INBUILD标准，预测因子是外周血中性粒细胞计数（OR = 2.06, 95% CI: 1.19-3.59; p = 0.01）和蜂蜂窝（OR = 69.1, 95% CI:2.16-2220; p = 0.02）。抗核抗体滴度对IPAF进展无影响。结论：纤维化HRCT特征（UIP，蜂窝状）和外周中性粒细胞增多是IPAF中ILD短期进展的预测因素。

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

Polskie Archiwum Medycyny Wewnetrznej-Polish Archives of Internal Medicine MEDICINE, GENERAL & INTERNAL-

CiteScore

4.50

自引率

0.00%

发文量

176

审稿时长

6-12 weeks

期刊介绍： Polish Archives of Internal Medicine is an international, peer-reviewed periodical issued monthly in English as an official journal of the Polish Society of Internal Medicine. The journal is designed to publish articles related to all aspects of internal medicine, both clinical and basic science, provided they have practical implications. Polish Archives of Internal Medicine appears monthly in both print and online versions.