Wang Huijuan, Wang Yuanying, Sun Di, Yu Shiwen, Du Xuqin, Ye Qiao
Clinical Center for Interstitial Lung Diseases, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Department of Occupational Medicine and Toxicology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Front Med (Lausanne). 2024 Jan 11;10:1325082. doi: 10.3389/fmed.2023.1325082. eCollection 2023.
Idiopathic inflammatory myopathy (IIM) frequently coexists with interstitial pneumonia (IP) and is commonly the initial or sole manifestation accompanied by positive myositis-specific autoantibodies (MSAs), even in the absence of meeting diagnostic criteria. This study aims to evaluate the proportion of progressive pulmonary fibrosis (PPF) and identify potential predictors influencing the progression of pulmonary fibrosis in patients with MSA-IP.
This descriptive study employed a retrospective cohort design, enrolling patients diagnosed with interstitial pneumonia and positive MSAs at Beijing Chao-Yang Hospital in a sequential manner. Clinical data were systematically collected from the patients' medical records during regular follow-up visits conducted every 3 to 6 months. Cox regression analysis was utilized to identify independent predictors of PPF in patients with positive MSAs and interstitial pneumonia.
A total of 307 patients were included in the study, with 30.6% of them developing PPF during a median follow-up period of 22 months. Kaplan-Meier survival curves demonstrated a significantly lower survival in the PPF patients compared to the non-PPF patients (median 11.6 months vs. 31 months, = 0.000). An acute/subacute onset of interstitial pneumonia (HR 3.231, 95%CI 1.936-5.392, = 0.000), lower diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted (HR 6.435, 95%CI 4.072-10.017, = 0.001), and the presence of diffuse alveolar damage (DAD) on high-resolution computed tomography (HRCT) (HR 8.679, 95%CI 1.974-38.157, = 0.004) emerged as independent predictors of PPF. Notably, the implementation of triple therapy comprising glucocorticoids, immunosuppressants, and antifibrotic drugs was associated with a reduced risk of developing PPF (HR 0.322, 95%CI 0.115-0.899, = 0.031).
Approximately 30.6% of patients with MSA-IP may develop PPF within the follow-up period. Patients presenting with an acute/subacute onset of interstitial pneumonia, lower predicted DLCO SB% and evidence of DAD on HRCT are more susceptible to developing PPF. Conversely, the administration of triple therapy appears to serve as a protective factor against the development of PPF in patients with MSA-IP.
特发性炎性肌病（IIM）常与间质性肺炎（IP）共存，即使未达到诊断标准，也通常是伴有肌炎特异性自身抗体（MSA）阳性的初始或唯一表现。本研究旨在评估进行性肺纤维化（PPF）的比例，并确定影响MSA-IP患者肺纤维化进展的潜在预测因素。
本描述性研究采用回顾性队列设计，连续纳入在北京朝阳医院诊断为间质性肺炎且MSA阳性的患者。在每3至6个月进行的定期随访期间，系统地从患者病历中收集临床数据。采用Cox回归分析确定MSA阳性和间质性肺炎患者PPF的独立预测因素。
本研究共纳入307例患者，其中30.6%在中位随访期22个月内发生PPF。Kaplan-Meier生存曲线显示，PPF患者的生存率显著低于非PPF患者（中位生存期11.6个月对31个月，P = 0.000）。间质性肺炎急性/亚急性起病（HR 3.231，95%CI 1.936 - 5.392，P = 0.000）、预测的肺一氧化碳弥散量（DLCO）%较低（HR 6.435，95%CI 4.072 - 10.017，P = 0.001）以及高分辨率计算机断层扫描（HRCT）显示存在弥漫性肺泡损伤（DAD）（HR 8.679，95%CI 1.974 - 38.157，P = 0.004）是PPF的独立预测因素。值得注意的是，采用糖皮质激素、免疫抑制剂和抗纤维化药物的三联疗法与发生PPF的风险降低相关（HR 0.322，95%CI 0.115 - 0.899，P = 0.031）。
约30.6%的MSA-IP患者在随访期内可能发生PPF。间质性肺炎急性/亚急性起病、预测的DLCO SB%较低以及HRCT显示有DAD证据的患者更容易发生PPF。相反，三联疗法的应用似乎是MSA-IP患者发生PPF的保护因素。
