Initial predictors for short-term prognosis in anti-melanoma differentiation-associated protein-5 positive patients

Abstract

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. Predictors of rapidly progressive ILD (RPILD) and poor outcomes were calculated using logistic regression models and Cox proportional hazard regression models, respectively. Results A total of 90 anti-MDA5 positive patients were included in this study. Eighty-one (90%) patients presented ILD on admission and 35 (38.9%) patients developed RPILD 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. Factors including disease duration 1600 U/ml (HR 3.7, 95% CI 1.5–9.1, P = 0.004) and CRP > 5.8 mg/L (HR 3.7, 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, initial intensive treatment may improve the prognosis.

Keywords

• Anti-melanoma differentiation-associated protein-5
• Interstitial lung disease
• Clinically amyopathic dermatomyositis
• Dermatomyositis