OBJECTIVE: To evaluate whether atherosclerotic cardiovascular disease outcomes, venous thromboembolism, heart failure, and cardiovascular mortality are elevated in patients with primary Sjögren's syndrome (pSS) compared with a matched cohort from the general population.

METHODS: We conducted a nationwide population-based cohort study including all patients with a pSS diagnosis from 1996 to 2017 in Denmark, matched with a comparison cohort on age, gender, and calendar year. The cumulative incidence of each outcome was computed using hospital contact diagnoses as the end point. We used Cox regression to calculate hazard ratios (HRs) for the endpoints, adjusted for potential confounding factors.

RESULTS: The study included 4697 pSS patients (median age 57 years, 87% women) and 46 970 persons in a comparison cohort followed for a median of 7.6 years (maximum 23 years). After confounder adjustment, pSS was associated with elevated rates of myocardial infarction (adjusted hazard ratio [aHR] 1.23; 95% confidence interval [CI] 1.01-1.50), ischaemic stroke (aHR 1.31; 95% CI 1.14-1.52) and haemorrhagic stroke (aHR 1.51; 95% CI 1.13-2.03), peripheral artery disease (aHR 1.44; 95% CI 1.13-1.83), and venous thromboembolism (aHR 1.57; 95% CI 1.33-1.85). Cardiovascular death (aHR 1.11; 95% CI 0.95-1.30) and heart failure (aHR 1.17; 95% CI 0.99-1.39) were slightly elevated in patients with pSS.

CONCLUSION: This study provides firm evidence that pSS is associated with increased risk of all atherosclerotic cardiovascular outcomes and venous thromboembolism, and highlights the importance of managing cardiovascular risk factors in these patients.