Justin Cooper , 2025-04-28 09:30:00
April 28, 2025
2 min read
Key takeaways:
- Mortality was significantly higher in patients with Sjögren’s vs. the general population, most commonly via infections.
- Key risk factors were age, C4 hypocomplementemia, elevated ESR, heart failure and lung involvement.
Mortality risk among patients with Sjögren’s disease is elevated as much as 70% vs. the general population, primarily due to infections and driven by heart and lung involvement, according to data published in The Journal of Rheumatology.
“When it comes to mortality in Sjögren’s disease, the picture isn’t so clear,” Olga Rusinovich, MD, a rheumatologist at Puerta de Hierro University Hospital, in Spain, told Healio. “Over the years, different studies have reported conflicting results — some suggesting that patients with Sjögren’s have only a slightly higher risk for death compared to the general population, while others point to a much greater risk, especially in those with systemic involvement.
“With such discrepancies in the data, we felt there was a real need to dig deeper,” she added. “By understanding these risks better, we hope to help clinicians make more informed decisions and provide patients with personalized, proactive care to improve long-term outcomes.”
To better assess these risks, Rusinovich and colleagues analyzed patients with Sjögren’s disease from a large, multicenter prospective cohort called SjögrenSER Prospective. The analysis included 314 patients (mean age, 66 years; 94.6% women) with an average disease duration of 17 years who were followed for a median period of 9.5 (interquartile range: 9.2-9.9) years.
Overall mortality was the primary outcome, with Cox proportional hazards models used to evaluate the associated patient characteristics. The researchers also calculated a standardized mortality ratio by comparing the observed deaths with expected deaths based on age- and sex-matched data from the Spanish general population.
Overall, 42 patients with Sjögren’s (13.4%) died during the study, according to the researchers. This yielded a standardized mortality ratio of 1.7 (95% CI, 1.2-2.3), meaning that the Sjögren’s mortality risk was 70% higher than the age- and sex-matched general population, the researchers wrote.
The most common causes of death were infections (35.7%), malignancies (23.8%) — particularly B-cell non-Hodgkin lymphoma, which resulted in 30% of malignancy-related deaths — and cardiovascular disease (7.1%).
“We knew infections were a concern, but we didn’t expect them to be the leading cause of death,” Rusinovich said. “This suggests that patients with Sjögren’s disease are particularly vulnerable to severe infection, possibly due to immune system dysfunction and the effects of immunosuppressive treatments.”
Multivariable Cox regression identified “five key risk factors” for mortality in Sjögren’s disease, according to Rusinovich. These were:
- Age, with each additional year of life increasing mortality risk by 11% (HR = 1.11; 95% CI, 1.08-1.15);
- C4 hypocomplementemia (HR = 3.75; 95% CI, 1.55-9.06);
- elevated erythrocyte sedimentation rate, with a 1% higher risk for every 1 mm/hour increase (HR = 1.01; 95% CI, 1-1.03);
- heart failure history (HR = 4.24; 95% CI, 1.02-17.58); and
- pulmonary involvement (HR = 3.31; 95% CI, 1.39-7.88).
Rusinovich said it was “interesting” that, unlike previous studies, mortality had no statistically significant relation to high disease activity and cryoglobulinemia.
“This could indicate that improvements in treatment and disease management have helped reduce their impact on survival,” she said, adding that “further studies are needed to confirm this.”
Rusinovich highlighted limitations of the study, including the lack of a direct control group, evolving treatments over time, and the drawing of patients from rheumatology units, where cases may be more severe.
“Even with these factors, one thing is clear: This study brings valuable insights that can help improve how we care for patients with Sjögren’s disease,” Rusinovich said.
For more information:
Olga Rusinovich, MD, can be reached at olga.rusinovich@gmail.com.
Sources/Disclosures
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Disclosures:
This study was funded by the Spanish Society of Rheumatology. The authors report no relevant financial disclosures.
