Qihua Yang1, Tianfang Li1, Xin Zhang1, Kunlong Lyu2, Shujun Wu3, Yan Chen4, Shengyun Liu5, Zujiang Yu6. 1. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. 2. Department of Urology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. 3. Department of Respiratory, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. 4. Department of Radiology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. 5. Department of Rheumatology and Immunology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. shengyun@medmail.com.cn. 6. Department of Infection Diseases, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China. johnyuem@zzu.edu.cn.
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 < 2 months (OR 6.1, 95% CI 1.7-22.4, P = 0.007), serum ferritin ≥ 1500 ng/ml (OR 12.3, 95% CI 3.1-49.6, P < 0.001), CRP ≥ 13 mg/L (OR 4.6, 95% CI 1.3-16.9, P = 0.021) and total GGO score ≥ 4 (OR 6.3, 95% CI 1.8-21.9, P = 0.003), were identified as independent predictors for RPILD. Cox regression model showed that total CT GGO score ≥ 4 (HR 4.8, 95% CI 1.3-17.9, P = 0.020), KL-6 > 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.
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 < 2 months (OR 6.1, 95% CI 1.7-22.4, P = 0.007), serum ferritin ≥ 1500 ng/ml (OR 12.3, 95% CI 3.1-49.6, P < 0.001), CRP ≥ 13 mg/L (OR 4.6, 95% CI 1.3-16.9, P = 0.021) and total GGO score ≥ 4 (OR 6.3, 95% CI 1.8-21.9, P = 0.003), were identified as independent predictors for RPILD. Cox regression model showed that total CT GGO score ≥ 4 (HR 4.8, 95% CI 1.3-17.9, P = 0.020), KL-6 > 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.
Authors: S A Greenberg; E M Bradshaw; J L Pinkus; G S Pinkus; T Burleson; B Due; L Bregoli; L S Bregoli; K C O'Connor; A A Amato Journal: Neurology Date: 2005-12-13 Impact factor: 9.910
Authors: M Infantino; M Manfredi; V Grossi; M Benucci; G Morozzi; E Tonutti; M Tampoia; N Bizzaro Journal: Clin Chim Acta Date: 2017-10-03 Impact factor: 3.786