Abstract
BackgroundInterstitial 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.ObjectiveThe 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.Methods333 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.ResultsRP-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.ConclusionsThere 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.
Highlights
idiopathic inflammatory myopathy (IIM) comprises a group of systemic autoimmune disorders, including DM, polymyositis, anti-synthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), affecting skeletal muscles and other organs
ASS patients with interstitial lung disease (ILD) generally have slower disease progression, another research from our study showed that anti-threonyl tRNA synthetase (PL-7), one of the subtypes of anti-aminoacyl-tRNA synthetase (ARS) antibodies, was closely associated with rapidly progressive interstitial lung disease (RP-ILD) [7]
Hyperferritinemia has been proven to be the hallmark and a predictor of poor outcome of ILD associated with melanoma differentiation-associated protein 5 (MDA5)+DM, different studies have different cut-off values of ferritin for prognosis [8, 9] and as far as we know, there is no separate study on ferritin in the RP-ILD population
Summary
IIM comprises a group of systemic autoimmune disorders, including DM, polymyositis, ASS, immune-mediated necrotizing myopathy (IMNM), inclusion body myositis (IBM), affecting skeletal muscles and other organs. There have been several studies that reported on the risk factor model of amyopathic dermatomyositis combined with ILD, which provides a favorable basis for better clinical assessment of disease progression [3, 4]. These studies did not separately discuss risk factors and prognosis of RP-ILD in MDA5+DM and ASS patients. 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 (RPILD) leading to poor prognosis
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