Sjögren disease carries a serious mortality risk that goes well beyond its hallmark symptoms of dry eyes and dry mouth. A large data linkage study out of Western Australia found that patients with primary Sjögren disease (SjD) face an 80% higher risk of death compared to matched individuals without the disease. Over 25 years, patients with SjD showed 23% lower relative survival than the general population, with infections, cardiovascular disease, and interstitial lung disease emerging as the leading causes of death.

The risk is compounded when SjD occurs alongside other conditions. Patients with SjD plus rheumatoid arthritis had a 2.1-fold higher risk of death, while those with SjD plus another connective tissue disease faced a 3.1-fold higher risk compared to controls. Interstitial lung disease showed the strongest individual association with death, and pulmonary circulation disorders carried a particularly elevated risk.

By the Numbers:

• 1.8x higher risk of death in primary SjD vs. controls
• 2.1x higher risk when SjD co-occurs with rheumatoid arthritis
• 3.1x higher risk when SjD co-occurs with other connective tissue diseases
• 5.4x increased risk of death from pulmonary circulation disorders
• 23% lower relative survival at 25 years vs. the general population

Why it matters: Clinicians often focus on symptom management in SjD, but this study highlights the importance of prioritizing cardiovascular risk factor management and infection prevention early in the disease course — steps that could meaningfully improve long-term survival.