A Clinical Risk Model to Predict Rapidly Progressive Interstitial Lung Disease Incidence in Dermatomyositis.

Yimin Li,Jingli Zhang,Zongxue Zhang,Yanying Liu,Xiaolin Sun,Xu Liu,Fuan Lin,Fangjingwei Xu,Yuhui Li,Yuguang Wang,Lianjie Shi

doi:10.3389/fmed.2021.733599

Abstract

Background: 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.Methods: 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.Results: 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.Conclusion: 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.

Highlights

Dermatomyositis is an autoimmune disease characterized by skin and muscle damage caused by muscular involvement and frequent extramuscular symptoms such as Raynaud’s phenomenon, arthritis, and interstitial lung disease (ILD) [1]
84.8% (140/165) of the patients in the development cohort showed ILD onset on CT, but they were unable to be diagnosed as rapidly progressive interstitial lung disease (RP-ILD) at that time
RP-ILD was diagnosed later, with the occurrence and development of the disease during 2 to 4 weeks of hospitalization, which was defined as a progressive deterioration of ILD in 3 months combining with rapidly progressive severe dyspnea and hypoxemia, requiring oxygen therapy or ventilator care [20, 21]

Summary

Introduction

Dermatomyositis is an autoimmune disease characterized by skin and muscle damage caused by muscular involvement and frequent extramuscular symptoms such as Raynaud’s phenomenon, arthritis, and interstitial lung disease (ILD) [1]. The prediction and timely identification of RP-ILD symptom onset is vital for effective treatment during early disease development stages and might lead to improved prognosis with significantly reduced mortality rates in patients with DM/CADM [11,12,13]. A variety of studies have explored baseline parameters associated with RP-ILD in patients with DM/CADM but few practical quantitative methods were established and validated in clinical practice to predict the incidence of RP-ILD. It is clinically significant to explore the RP-ILD-associated parameters at the onset of DM/CADM to establish a reliable early-stage RP-ILD risk prediction (RRP) model. 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

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Conclusion