Background/Purpose: Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands with potential systemic involvement. Despite its known complications, population-level mortality trends in Sjögren’s syndrome remain poorly characterized. This study evaluates national mortality trends and sociodemographic disparities in Sjögren’s syndrome-related deaths in the United States from 1999 to 2020.

Methods: We used the CDC WONDER Multiple Cause of Death database to identify decedents with ICD-10 code M35.0 (Sjögren’s syndrome) listed as a cause of death from 1999 to 2020. Age-adjusted mortality rates (AAMRs) were calculated per 100,000 population using the direct method and the 2000 U.S. standard population. Mortality trends were analyzed over time and stratified by sex, age, race/ethnicity, urbanization, place of death, and U.S. state of residence.

Results: A total of 2,149 Sjögren’s syndrome-related deaths occurred during the study period. The AAMR increased by 57.5%, from 0.0202 in 1999 to 0.0321 in 2020. Females accounted for 88.2% of deaths and had an AAMR over eight times higher than males (0.049 vs. 0.006). Mortality increased markedly with age, from 0.01 per 100,000 in adults aged 45–54 to 0.43 in those aged ≥85 years. By race/ethnicity, White individuals had the highest AAMR (0.028), followed by American Indian/Alaska Native (0.032), Black (0.013), Asian/Pacific Islander (0.014), and Hispanic individuals (0.019). AAMRs rose with increasing rurality, ranging from 0.0202 in large central metro areas to 0.0278 in noncore rural regions. State-level AAMRs varied widely, from 0.015 in Connecticut to 0.051 in Washington. The majority of deaths occurred in inpatient hospital settings (42.3%) and nursing homes or long-term care facilities (26.6%), followed by the decedent’s home (23.9%), hospice facilities (1.9%), and emergency/outpatient departments (2.6%).

Conclusion: Mortality from Sjögren’s syndrome in the United States has increased over the past two decades, with a disproportionate burden among elderly women, rural populations, and White individuals. These findings underscore the need for improved disease recognition, timely systemic management, and targeted interventions to address healthcare disparities in Sjögren’s syndrome.

Graphs showing state, urbanization, gender, race, place of death, age and year.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/silent-impact-two-decades-of-sjogrens-syndrome-mortality-in-the-united-states/