RT Journal Article
SR Electronic
T1 Clinical Course, Prognosis, and Causes of Death in Mixed Connective Tissue Disease
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1134
OP 1142
DO 10.3899/jrheum.121272
VO 40
IS 7
A1 Agota Hajas
A1 Peter Szodoray
A1 Britt Nakken
A1 Janos Gaal
A1 Eva Zöld
A1 Renata Laczik
A1 Nora Demeter
A1 Gabor Nagy
A1 Zoltan Szekanecz
A1 Margit Zeher
A1 Gyula Szegedi
A1 Edit Bodolay
YR 2013
UL http://www.jrheum.org/content/40/7/1134.abstract
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.