Initial Predictors for Short-termprognosisin Anti-melanoma Differentiation-associated Protein-5 Positive Patients

Abstract

Background: Anti-melanoma differentiation-associated protein-5 (anti-MDA5) positive patients are characterized by the high mortality rate caused by interstitial lung disease (ILD). We conducted a retrospective study to summarize the clinical features and identify the initial predictors for death in anti-MDA5 positive patients.Methods:We designed a retrospective cohort of anti-MDA5 positive patients.The demographic and clinical data recorded on first admission, as well as the outcomes during the first six months follow-up were collected. Risk factors for death were identified using multivariate analyses.Results: A total of 90 anti-MDA5 positive patients were included in this study. Eighty-one (90%) patients presentedILD on admission and 35(38.9%) patients developed rapidly progressive ILD (RP-ILD) subsequently.During the first six months of follow-up, 22 (24.4%) patients died of respiratory failure at an average time of 6.6 ± 5.9 weeks.Univariate analysis identified several factors associated with death, including demographic, clinical, laboratory and image variables. Multivariate analysis showed that total CT GGO score≥4(HR 4.8, 95%CI 1.3-17.9,P=0.020), KL-6>1600U/ml (HR3.7, 95%CI 1.5-9.1, P=0.004) and CRP>5.8mg/L (HR3.7, 95%CI 1.0-12.8, P=0.044) were poor prognostic risk factors, however initial combined treatment(HR 0.3, 95%CI 0.1-0.8, P=0.019) predicted good prognosis in anti-MDA5 positive patients.Conclusion: Anti-MDA5 positive patients demonstrated a high prevalence of ILD on admission, leading to a high short-term mortality rate. Higher total GGO score, higher levels of initial KL-6 and CRP predict poor outcome in anti-MDA5 positive patients. However, in tial intensive treatment may improve the prognosis.