结缔组织病相关间质性肺疾病呈进展性纤维化表型的临床特征及危险因素分析

Clinical characteristics and risk factors of connective tissue disease associated interstitial lung disease with progressive fibrosis phenotype

摘要:

目的 探讨结缔组织病相关间质性肺疾病（CTD-ILD）患者呈进展性肺纤维化表型的临床特点及危险因素，以期为疾病的早期诊断提供理论依据。方法 回顾性分析2015年11月—2019年3月桂林医学院附属医院诊断为CTD-ILD的127例患者的临床资料。其中，呈进展性肺纤维化表型41例为PF-ILD组，非进展性纤维化表型86例为non-PF-ILD组。比较两组患者的人口学特征及病种构成、临床表现、实验室指标和肺部高分辨率CT影像特征；影响因素的分析采用多因素Logistic回归模型；绘制受试者工作特征（ROC）曲线评估Logistic回归模型的准确性。结果 CTD-ILD患者中呈进展性肺纤维化表型的发生率为32.3%，疾病构成主要包括多发性肌炎/皮肌炎、类风湿性关节炎、系统性硬化症、干燥综合征。与non-PF-ILD组患者比较，PF-ILD组患者病程缩短（P <0.05），咳嗽、气促占比增加（P <0.05），白细胞计数、中性粒细胞比值、红细胞沉降率、C反应蛋白升高（P <0.05），血清白蛋白水平降低（P <0.05）。同时，PF-ILD组患者IgA水平升高（P <0.05），B淋巴细胞的比例下降，CEA、CA199水平升高（P <0.05），HRCT影像特征呈蜂窝状改变例数增加（P <0.05）。IgA水平升高、CA199水平升高和HRCT呈蜂窝状改变是CTD-ILD患者出现进展性肺纤维化的独立危险因素（P <0.05）。ROC曲线对该Logistic回归模型评价结果显示，其曲线下面积为0.818（95% CI：0.735，0.884），约登指数为0.522，敏感性为57.50%（95% CI：0.451，0.682），特异性为94.67%（95% CI：0.905，0.977）。结论 IgA水平和CA199水平升高及HRCT呈蜂窝状改变是CTD-ILD患者出现进展性肺纤维化的独立危险因素。

Abstract:

Objective To explore the clinical characteristics and risk factors of the progressive pulmonary fibrosis phenotype in patients with connective tissue disease-related interstitial lung disease (CTD-ILD) and provide theoretical evidence for the early diagnosis of the disease.Methods A retrospective analysis was conducted on clinical data from 127 patients diagnosed with CTD-ILD between November 2015 and March 2019 at the Affiliated Hospital of Guilin Medical College. Of these patients, 41 presented with a progressive pulmonary fibrosis phenotype (PF-ILD group), and 86 had a non-progressive fibrosis phenotype (non-PF-ILD group). Comparative analyses were performed regarding demographic characteristics, disease spectrum, clinical manifestations, laboratory indicators, and high-resolution CT (HRCT) imaging features between the two groups. Multivariate logistic regression models were used to analyze influencing factors, and the receiver operating characteristic (ROC) curve was generated to evaluate the accuracy of the logistic regression model.Results The incidence of the progressive pulmonary fibrosis phenotype among CTD-ILD patients was 32.3%. The disease spectrum mainly included polymyositis/dermatomyositis, rheumatoid arthritis, systemic sclerosis, and Sj?gren's syndrome. Compared to the non-PF-ILD group, the PF-ILD group had a shorter disease duration (P < 0.05), increased cough and dyspnea (P < 0.05), elevated white blood cell count, neutrophil ratio, erythrocyte sedimentation rate, and C-reactive protein (P < 0.05), and decreased serum albumin levels (P < 0.05). Additionally, the PF-ILD group showed higher IgA levels (P < 0.05), a decrease in B lymphocyte proportions, and elevated CEA and CA199 levels (P < 0.05). HRCT imaging features demonstrated an increased number of honeycomb changes (P < 0.05) in the PF-ILD group. Elevated IgA and CA199 levels, along with HRCT displaying honeycomb changes, were independent risk factors for the development of progressive pulmonary fibrosis in CTD-ILD patients (P < 0.05). The ROC curve for this logistic regression model had an area under the curve of 0.818 (95% CI: 0.735, 0.884), a Youden index of approximately 0.522, a sensitivity of 57.50% (95% CI: 0.451, 0.682), and a specificity of 94.67% (95% CI: 0.905, 0.977).Conclusion Elevated IgA and CA199 levels, along with HRCT imaging displaying honeycomb changes, are independent risk factors for the development of progressive pulmonary fibrosis in CTD-ILD patients.