Journal Article

Different Multivariable Risk Factors for Rapid Progressive Interstitial Lung Disease in Anti-MDA5 Positive Dermatomyositis and Anti-Synthetase Syndrome

Chen, Fang, Wang, Guochun, Shen, Yawen, Ye, Lifang, Zuo, Yu, Lu, Xin, Shu, Xiaoming

2022

Overview

Interstitial lung disease (ILD) is frequently observed in anti-melanoma differentiation-associated protein 5 (MDA5) antibody positive dermatomyositis (DM) and anti-synthetase syndrome (ASS), where they often develop a rapidly progressive ILD (RP-ILD) leading to poor prognosis. The aim of this study was to construct multivariable prediction risk factors for rapid progressive ILD (RP-ILD) in anti-MDA5 positive DM (MDA5 DM) and ASS. 333 idiopathic inflammatory myopathy (IIM) associated ILD patients were studied retrospectively. Risk factors for RP-ILD in MDA5 DM and ASS patients were identified by univariate and multivariable logistic regression analysis. The mortality was assessed using Kaplan-Meier analysis. RP-ILD was more prevalent in MDA5 DM patients than ASS patients. MDA5 DM patients with RP-ILD had significantly lower survival rates than those in ASS patients. The independent risk factors for RP-ILD in MDA5 DM patients were fever (OR 3.67, 95% CI:1.79-7.52), lymphopenia (OR 2.14, 95% CI:1.01-4.53), especially decreased levels of CD3 T cells (OR 2.56, 95% CI:1.17-5.61), decreased levels of CD3 CD4 T cells (OR 2.80, 95% CI:1.37-5.73), CD3 CD8 T cells (OR 2.18, 95% CI:1.05-4.50), elevated CD5 CD19 B cells (OR 3.17, 95% CI:1.41-7.13), elevated ALT (OR 2.36, 95% CI:1.15-4.81), high lactate dehydrogenase (LDH) (OR 3.08, 95% CI:1.52-6.27), hyper-ferritin (OR 4.97, 95% CI:1.97-12.50), elevated CEA (OR 2.28, 95% CI:1.13-4.59), and elevated CA153 (OR 3.31, 95% CI:1.50-7.27). While the independent risk factors for RP-ILD in ASS patients were elevated CEA (OR 5.25, 95% CI: 1.73-15.93), CA125 (OR 2.79, 95% CI: 1.10-7.11) and NSE (OR 4.86, 95% CI: 1.44-16.37). Importantly, serum ferritin>2200ng/ml predicted patient's death within half a year in MDA5 DM patients with RP-ILD, but not in ASS patients. There were significant different mortality and multivariable risk factors for RP-ILD in MDA5 DM patients and ASS patients. Potential clinical benefits of using these different risk factors deserve assessment of severity and prognosis in IIM patients.

• Publisher: Frontiers Media SA,Frontiers Media S.A
• Subject: anti-MDA5 antibody / anti-synthetase syndrome / Antibodies / Autoantibodies / Carbon monoxide / Carcinoembryonic Antigen / CD19 antigen / CD3 antigen / CD4 antigen / CD5 antigen / CD8 antigen / Demographics / Dermatomyositis / Disease Progression / Ferritin / Ferritins / Humans / hyperferritinemia / idiopathic inflammatory myopathy / Immunology / L-Lactate dehydrogenase / Lavage / Ligases / Lung diseases / Lung Diseases, Interstitial - etiology / Lymphocytes / Lymphocytes B / Lymphocytes T / Lymphopenia / Medical prognosis / Melanoma / Mortality / Myopathy / Myositis - complications / Patients / Prognosis / rapidly progressive interstitial lung disease / Retrospective Studies / Risk Factors / Statistical analysis / Steroids / Syndrome / Transfer RNA