Abstract
Objective To investigate the association of distinct myositis specific autoantibodies (MSAs) with long-term survival of patients with polymyositis (PM) and dermatomyositis (DM). Methods We analyzed the clinical data and outcome of patients with PM and DM who were hospitalized in the department of rheumatology of China-Japan Friendship hospital from 1994 to 2015, and evaluated the impact of MSAs on the prognosis of patients. Multivariate Cox regression analysis was used to identify the prognostic risk factors for PM/DM patients. Results A total of 383 PM/DM patients were followed up for 1-333 months. Cumulative survival and 10-year survival rate of all patients were 68.6% and 76.2%, respectively. The survival rate of 80.4% and 77.1% at 3 and 5 years in patients with MSAs, which were lower than those of patients with-out MSAs, who had the survival rate of 90.1% and 87.4% at 3 and 5 years, respectively(χ2=3.90 and 3.98, P<0.05). There was significant difference for long-term survival in all MSAs positive groups (χ2=40.654, P=0.000). Anti-MDA5 positive patients who had the 10-year survival rate of 28.7% had the worst prognosis, while anti-HMGCR positive patients who had the 10-year survival rate of 100% had the best outcome in all groups. Multivariate Cox regression analysis showed that independent risk factors associated with the long-term survival of patients were age of onset, complicated with malignancies, dysphagia, rapidly progress interstitial lung disease, anti-MDA5 antibody positive, increased serum aspartate transferase and C reaction protein. Conclusion MSAs are strongly associated with the prognosis of patients with PM/DM. Patients with MSAs has worse 5-year overall survival than those without MSAs, which indicates that screening MSAs and aggressive treatment for PM/DM patients at very early stage of disease may improve the outcome. Key words: Polymyositis; Dermatomyositis; Autoantibodies; Survival
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