title: Clinical course, prognosis, and causes of death in mixed connective tissue disease.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: Division of Clinical Immunology, Department of Medicine, Medical and Health Science Centre, University of Debrecen, Debrecen, Hungary.
authors: Agota
authors: A
authors: Hajas
authors: Peter
authors: P
authors: Szodoray
authors: Britt
authors: B
authors: Nakken
authors: Janos
authors: J
authors: Gaal
authors: Eva
authors: E
authors: Zöld
authors: Renata
authors: R
authors: Laczik
authors: Nora
authors: N
authors: Demeter
authors: Gabor
authors: G
authors: Nagy
authors: Zoltan
authors: Z
authors: Szekanecz
authors: Margit
authors: M
authors: Zeher
authors: Gyula
authors: G
authors: Szegedi
authors: Edit
authors: E
authors: Bodolay
mesh_terms: D000328:Adult; D000368:Aged; D017153:Antibodies, Anticardiolipin / Q000276:immunology*; D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D002423:Cause of Death; D018450:Disease Progression; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis* / Q000276:immunology / Q000401:mortality*; D011379:Prognosis; D015996:Survival Rate
chemical_list: D017153:Antibodies, Anticardiolipin; D017152:Antibodies, Antiphospholipid
keywords: ANTIPHOSPHOLIPID SYNDROME; CARDIOVASCULAR EVENTS; CLINICAL COURSE; MIXED CONNECTIVE TISSUE DISEASE; PROGNOSIS; SURVIVAL