PT - JOURNAL ARTICLE AU - Agota Hajas AU - Peter Szodoray AU - Britt Nakken AU - Janos Gaal AU - Eva Zöld AU - Renata Laczik AU - Nora Demeter AU - Gabor Nagy AU - Zoltan Szekanecz AU - Margit Zeher AU - Gyula Szegedi AU - Edit Bodolay TI - Clinical Course, Prognosis, and Causes of Death in Mixed Connective Tissue Disease AID - 10.3899/jrheum.121272 DP - 2013 May 01 TA - The Journal of Rheumatology PG - jrheum.121272 4099 - http://www.jrheum.org/content/early/2013/04/25/jrheum.121272.short 4100 - http://www.jrheum.org/content/early/2013/04/25/jrheum.121272.full AB - Objective To study the survival rate and prognostic indicators of mixed connective tissue disease (MCTD) in a Hungarian population. Methods Two hundred eighty patients with MCTD diagnosed between 1979 and 2011 were followed prospectively. Clinical features, autoantibodies, and mortality data were assessed. Prognostic factors for survival were investigated and survival was calculated from the time of the diagnosis by Kaplan-Meier method. Results A total of 22 of 280 patients died: the causes of death were pulmonary arterial hypertension (PAH) in 9 patients, thrombotic thrombocytopenic purpura in 3, infections in 3, and cardiovascular events in 7. The 5, 10, and 15-year survival rates after the diagnosis was established were 98%, 96%, and 88%, respectively. The deceased patients were younger at the diagnosis of MCTD compared to patients who survived (35.5 ± 10.4 vs 41.8 ± 10.7 yrs; p < 0.03), while there was no difference in the duration of the disease (p = 0.835). Our cohort study showed that the presence of cardiovascular events (p < 0.0001), esophageal hypomotility (p = 0.04), serositis (p < 0.001), secondary antiphospholipid syndrome (p = 0.039), and malignancy (p < 0.001) was significantly higher in the deceased patients with MCTD. The presence of anticardiolipin (p = 0.019), anti-β2-glycoprotein I (p = 0.002), and antiendothelial cell antibodies (p = 0.002) increased the risk of mortality. Conclusion Overall, PAH remained the leading cause of death in patients with MCTD. The prevalence of cardiovascular morbidity and mortality, malignancy, and thrombotic events increased during the disease course of MCTD. The presence of antiphospholipid antibodies raised the risk of mortality.