Increased risk of aortic aneurysm and dissection in patients with Sjögren's syndrome: a nationwide population-based cohort study in Taiwan

BMJ Open. 2018 Sep 21;8(9):e022326. doi: 10.1136/bmjopen-2018-022326.


Objectives: Sjögren's syndrome (SS) is a systemic autoimmune disorder. Several molecular pathways and the activation of matrix metalloproteinases associated with the pathogenesis of SS participate in the initiation and progression of aortic aneurysm (AA) and aortic dissection (AD). In this study, we aimed to evaluate whether patients with SS exhibit an increased risk of AA or AD.

Methods: We conducted a retrospective cohort study using a database extracted from Taiwan's National Health Insurance Research Database. All medical conditions for each case and control were categorised using the International Classification of Diseases, Ninth Revision. HRs and 95% CIs for associations between SS and AA/AD were estimated using Cox regression and adjusted for comorbidities.

Results: Our analyses included 10 941 SS cases and 43 764 propensity score-matched controls. Compared with the controls, the patients with SS exhibited a significantly increased risk of developing an AA or AD (adjusted HR=3.642, p<0.001). Subgroup analysis revealed that compared with patients without SS, patients with primary and secondary SS both exhibited a significantly increased risk of developing AA or AD (adjusted HR=1.753, p=0.042; adjusted HR=3.693, p<0.001).

Conclusion: Patients with SS exhibit increased risks of developing AA or AD, and healthcare professionals should be aware of this risk when treating patients with SS. Increased aortic surveillance may be required for patients with SS.

Keywords: Sjögren’s syndrome; aortic aneurysm; aortic dissection.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aortic Aneurysm* / diagnosis
  • Aortic Aneurysm* / epidemiology
  • Aortic Dissection* / epidemiology
  • Aortic Dissection* / prevention & control
  • Cohort Studies
  • Databases, Factual / statistics & numerical data
  • Disease Progression
  • Early Diagnosis
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Retrospective Studies
  • Risk Factors
  • Sjogren's Syndrome* / epidemiology
  • Sjogren's Syndrome* / physiopathology
  • Taiwan / epidemiology