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

• DOI: 10.1186/s13023-021-01705-8
• Publication Date: 2021-01-30T13:02:52Z
• Authors: Qihua Yang; Tianfang Li; Xin Zhang; Kunlong Lyu; Shujun Wu; Yan Chen; Shengyun Liu; Zujiang Yu
• Keywords: 0301 basic medicine; Interferon-Induced Helicase, IFIH1; Research; R; Interstitial lung disease; Anti-melanoma differentiation-associated protein-5; Prognosis; Dermatomyositis; 3. Good health; 03 medical and health sciences; Clinically amyopathic dermatomyositis; 0302 clinical medicine; Disease Progression; Medicine; Humans; 10. No inequality; Autoantibodies; Retrospective Studies

Abstract Background Anti-melanoma differentiation-associated protein-5 (anti-MDA5) positive patients are characterized by the high mortality rate caused interstitial lung disease (ILD). We conducted a retrospective study to summarize clinical features and identify initial predictors for death in anti-MDA5 patients. Methods designed cohort of The demographic data recorded on first admission, as well outcomes during six months follow-up, were collected. Predictors rapidly progressive ILD (RPILD) poor calculated using logistic regression models Cox proportional hazard models, respectively. Results A total 90 included this study. Eighty-one (90%) presented admission 35 (38.9%) developed RPILD subsequently. During 22 (24.4%) died respiratory failure at an average time 6.6 ± 5.9 weeks. Factors including duration < 2 (OR 6.1, 95% CI 1.7–22.4, P = 0.007), serum ferritin ≥ 1500 ng/ml 12.3, 3.1–49.6, 0.001), CRP 13 mg/L 4.6, 1.3–16.9, 0.021) GGO score 4 6.3, 1.8–21.9, 0.003), identified independent RPILD. model showed that CT (HR 4.8, 1.3–17.9, 0.020), KL-6 > 1600 U/ml 3.7, 1.5–9.1, 0.004) 5.8 1.0–12.8, 0.044) prognostic risk factors, however combined treatment 0.3, 0.1–0.8, 0.019) predicted good prognosis Conclusion Anti-MDA5 demonstrated prevalence leading short-term rate. Higher score, higher levels predict outcome However, intensive may improve prognosis.