Objectives: We studied a cohort of Sjögren’s syndrome patients followed at a single centre for over 35 years, analyze their outcomes during the course of the disease.

Methods: A cohort of 232 patients with primary Sjögren’s syndrome was retrospectively assessed at University College London Hospital, UK, over a very long period to capture the evolving features and complications of the disease and to focus on the causes of death.

Results: The mean age at diagnosis was 50.5 years (SD=14.78 years). The mean years of follow-up since diagnosis was 12.1 years (SD= 8.65); 48 patients (20.7%) were lost to follow-up at some point. Lymphoma developed in 20 patients. When compared with the rest of the patients, the incidence of glandular manifestations was higher in the lymphoma group (11 [55%] vs 59 [37.8%]; p=0.005). In independent multivariate regression analysis, parotid swelling at the time of diagnosis was the most important predictive factor. We compared patients between <15 and >15 years of follow-up and found a statistically significant difference in arthritis (p=0.010), Raynaud’s phenomenon (p ≤ 0.001), and vasculitis (p=0.013). The majority of lymphomas developed <15 years post-diagnosis (8.6% vs 5.5%). Thirty-seven patients (20%) died, with a mean age of 80.20 years old (SD=8.547); infection was the main cause of death (11 out of 24 causes of death were identified).

Conclusion: This very long-term follow-up (>35 years) shows that the main complication associated with Sjögren’s syndrome was related to lymphoma, which tends to develop during the first years of the disease. In contrast, in our cohort among those patients who died, the majority were over 70 years old (mean age of death was 80.20 years), confirming that the disease is clearly compatible with a long-life expectancy.

Sjogren’s syndrome, Long-term effects, Follow-up studies, Lymphoma