Koh Jung Hee, Park Youngjae, Lee Jennifer, Jeon Howook, Moon Su-Jin, Kim Yong Hyun, Min Jun-Ki, Park Sung-Hwan, Kwok Seung-Ki
Division of Rheumatology, Department of Internal Medicine, Uijeongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Korean J Intern Med. 2025 Jan;40(1):148-159. doi: 10.3904/kjim.2023.402. Epub 2024 Oct 25.
BACKGROUND/AIMS: Interstitial lung disease (ILD) is a potentially serious but underdiagnosed manifestation of primary Sjögren's syndrome (pSS). This observational study investigated the prevalence and clinical course of ILD in pSS, together with prognostic factors.
A multicenter, retrospective longitudinal study was performed using findings from baseline and follow-up pulmonary function tests and chest computed tomography. Predisposing factors for the development of ILD and acute exacerbation (AE) were identified using a logistic regression model. The risk factors for a significant decline of pulmonary function were determined by the Cox proportional hazard model.
A total of 1,306 patients with pSS were included in this study (female, 98%; mean age, 54 years). Among them, 79 patients with pSS were comorbid with ILD. ILD was more frequently found in male, older patients. Nonspecific interstitial pneumonia was the most prevalent imaging pattern in pSS-ILD (51%), followed by usual interstitial pneumonia (22%). At diagnosis with pSS-ILD, 54% of patients had restrictive pulmonary function, and 41% of patients initiated pharmacological treatment. During the median 4-year follow-up period, AE, a significant decline in pulmonary function, and death occurred in 19%, 29%, and 9% of patients with pSS-ILD, respectively. The neutrophil-to-lymphocyte ratio (NLR) increased 3 months prior to AE, and it was associated with AE. Older age at pSS-ILD diagnosis was a prognostic factor for a significant decline in pulmonary function.
ILD accounted for 6% of the comorbidity of pSS. AE was associated with a significant decline in pulmonary function, and the NLR may predict AE.
背景/目的：间质性肺疾病（ILD）是原发性干燥综合征（pSS）一种潜在严重但诊断不足的表现。本观察性研究调查了pSS中ILD的患病率、临床病程以及预后因素。
利用基线和随访肺功能测试及胸部计算机断层扫描结果进行了一项多中心回顾性纵向研究。使用逻辑回归模型确定ILD和急性加重（AE）发生的易感因素。通过Cox比例风险模型确定肺功能显著下降的危险因素。
本研究共纳入1306例pSS患者（女性占98%；平均年龄54岁）。其中，79例pSS患者合并ILD。ILD在男性、老年患者中更常见。非特异性间质性肺炎是pSS-ILD中最常见的影像学表现（51%），其次是寻常型间质性肺炎（22%）。在诊断为pSS-ILD时，54%的患者有限制性肺功能，41%的患者开始药物治疗。在中位4年的随访期内，pSS-ILD患者中分别有19%、29%和9%发生AE、肺功能显著下降和死亡。中性粒细胞与淋巴细胞比值（NLR）在AE前3个月升高，且与AE相关。pSS-ILD诊断时年龄较大是肺功能显著下降的预后因素。
ILD占pSS合并症的6%。AE与肺功能显著下降相关，NLR可能预测AE。
