Overall survival and mortality risk factors in Takayasu's arteritis: A multicenter study of 318 patients

J Autoimmun. 2019 Jan:96:35-39. doi: 10.1016/j.jaut.2018.08.001. Epub 2018 Aug 17.


Objective: To report the long term mortality in Takayasu arteritis (TA) and to identify prognosis factors.

Methods: We analyzed the causes of death and the factors associated with mortality in a cohort of 318 patients [median age at diagnosis was 36 [25-47] years and 276 (86%) patients were women] fulfilling American College of Rheumatology and/or Ishikawa criteria of TA. A prognostic score for death and vascular complications was elaborated based on a multivariate model.

Results: Among 318 TA patients, 16 (5%) died after a median [IQR] follow-up of 6.1 [2.8-13.0] years. The median age at death was 38 [25-47] years with 88% of women. Main causes of death included mesenteric ischemia (n = 4, 25%) and aortic aneurysm rupture (n = 4, 25%). The mortality rate at 5 and 10 years was of 1.9% and 3.9%, respectively. Caucasians (p = 0.049) and smokers (p = 0.002) TA patients were more likely to die. There was an increased mortality in TA (SMR with 95% confidence interval, 2.73 [1.69-4.22]) as compared to age and sex matched healthy controls. We defined high risk patients for death and vascular complications according to the presence of two of the following factors (i.e a progressive clinical course, thoracic aorta involvement and/or retinopathy). In the high risk TA group, the 5-year incidence of death and vascular complication was 48.5% compared to 21.6% (p = 0.001) in those with low risk.

Conclusion: The overall mortality in our Takayasu cohort was 5% after a median follow-up of 6.1 years. We identified specific characteristics that distinguish TA patients at highest risk for death and vascular complications.

Keywords: Epidemiology; Mortality risk factors; Takayasu arteritis; Vasculitis.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aortic Rupture
  • Cigarette Smoking
  • Disease Progression
  • Female
  • Follow-Up Studies
  • France / epidemiology
  • Humans
  • Incidence
  • Male
  • Mesenteric Ischemia
  • Middle Aged
  • Risk Factors
  • Sex Factors*
  • Survival Analysis
  • Takayasu Arteritis / epidemiology*
  • Takayasu Arteritis / mortality
  • White People*