Li Yimin, Li Yuhui, Wang Yuguang, Shi Lianjie, Lin Fuan, Zhang Zongxue, Zhang Jingli, Liu Yanying, Liu Xu, Xu Fangjingwei, Sun Xiaolin
Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis, Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Department of Respiratory, Beijing Hospital of Traditional Chinese Medicine, Beijing, China.
Front Med (Lausanne). 2021 Sep 27;8:733599. doi: 10.3389/fmed.2021.733599. eCollection 2021.
Rapidly progressive interstitial lung disease (RP-ILD) is a fatal complication of dermatomyositis (DM) and clinically amyopathic DM (CADM). The objective of this study was to evaluate risk markers associated with RP-ILD incidence in patients with DM/CADM and to develop a RP-ILD risk prediction (RRP) model. The clinical records of 229 patients with DM/CADM from Peking University People's Hospital, and 97 patients from four other independent clinical centers were retrospectively reviewed. Univariate and multivariate logistic regression analyses were performed to identify independent risk factors associated with later RP-ILD incidence to build a risk score model. The concordance index (C-index) and calibration curve were calculated to evaluate the predictive accuracy of the RRP model. A multiparametric RRP model was established based on weighted clinical features, including fever (yes, 5; no, 0), periungual erythema (yes, 6; no, 0), elevated CRP (yes, 5; no, 0), anti-MDA5 antibody (positive, 8; negative, 0), and anti-Ro-52 antibody (positive, 6; negative, 0). Patients were divided into three risk groups according to the RRP total score: low, 0-9; medium, 10-19; high, 20-30. The C-index and calibration curve of the RRP model showed a promising predictive accuracy on the incidence of RP-ILD. The RRP model might promisingly predict the incidence of RP-ILD in DM/CADM patients to guide early individual treatment and further improve the prognosis of DM/CADM patients.
快速进展性间质性肺疾病（RP-ILD）是皮肌炎（DM）和临床无肌病性皮肌炎（CADM）的一种致命并发症。本研究的目的是评估DM/CADM患者中与RP-ILD发病相关的风险标志物，并建立一个RP-ILD风险预测（RRP）模型。回顾性分析了北京大学人民医院229例DM/CADM患者以及其他四个独立临床中心97例患者的临床记录。进行单因素和多因素逻辑回归分析，以确定与后期RP-ILD发病相关的独立危险因素，从而建立风险评分模型。计算一致性指数（C指数）和校准曲线，以评估RRP模型的预测准确性。基于加权临床特征建立了一个多参数RRP模型，包括发热（是，5分；否，0分）、甲周红斑（是，6分；否，0分）、CRP升高（是，5分；否，0分）、抗MDA5抗体（阳性，8分；阴性，0分）和抗Ro-52抗体（阳性，6分；阴性，0分）。根据RRP总分将患者分为三个风险组：低风险组，0-9分；中风险组，10-19分；高风险组，20-30分。RRP模型的C指数和校准曲线显示出对RP-ILD发病率有良好的预测准确性。RRP模型有望预测DM/CADM患者中RP-ILD的发病率，以指导早期个体化治疗，并进一步改善DM/CADM患者的预后。
