Background/Purpose: Systemic sclerosis is one of the most fatal rheumatologic diseases, with significantly higher mortality rates compared to the general population, likely due to its fibrotic nature and consequential effects on the vasculature system. Although previous observational population-based studies looked into the mortality rates of this disorder, more recent research beyond 2020 is limited, especially analyses stratified by demographic and regional distributions in the United States.

Methods: Datasets from the CDC WONDER Multiple Cause-of-Death public use records from 1999 to 2023 were used to analyze deaths in adults ≥25 years old assigning ICD-10 codes (M34 series) to systemic sclerosis by year and place of death, as well as by demographic (sex, age group, race) and regional (state, census region, rural-urban) stratifications. Age-adjusted mortality rates (AAMR) were calculated per 1,000,000 with 95% confidence intervals by standardizing crude mortality rates (CMR). Annual percent change (APC) and average annual percent change (AAPC) were evaluated using Joinpoint regression software.

Results: 44,407 deaths occurred from 1999 to 2023, mostly in medical facilities (57.3%). AAMR declined from 10.8 to 6.3 in 2018, followed by stability until 5.8 in 2023 (AAPC: -2.4; 95%CI: -2.8 to -2.2). Women had a higher average AAMR compared to men, along with a lower rate of decrease (AAPC women: -2.3; 95%CI: 2.6 to -2.0, men: -2.6; 95%CI: -3.4 to -2.1). Adults ≥65 years old had the highest average AAMR along with the lowest rate of decrease amongst the age groups (AAPC ≥65 years old: -2.0; 95%CI: -2.4 to -1.6, 45-65 years old: -3.3: 95%CI: -3.6 to -3.0, 25-44 years old: -3.2; 95%CI: -3.9 to -2.6). NH Black/African Americans had the highest average AAMR, but had had the highest rate of decrease (AAPC NH Black/African American: -3.1; 95%CI: -3.5 to -2.7, NH White: -2.4; 95%CI -2.9 to -2.1, NH Asian/Pacific Islander: -2.0; 95%CI: -2.7 to -1.3, Hispanic/Latino: -2.3; 95%CI -3.5 to -1.2). Regionally, the Midwest had the highest average AAMR, though the South had the highest rate of decrease (AAPC South: -2.9; 95%CI: -3.4 to -2.5, West: -2.4; 95%CI: -2.9 to -2.0, Midwest: -2.3; 95%CI: -2.6 to -2.0, Northeast: -1.9; 95%CI: -2.4 to -1.6). From 1999 to 2020, AAMR extended from 4.8 in Nevada to 13.6 in South Dakota. From 2021 to 2023, AAMR extended from 3.3 in Alabama to 9.9 in Montana. From 1999-2020, urban areas had higher average AAMR, with a higher rate of decrease (AAPC urban: -2.9; 95%CI: -3.1 to -2.7, rural: -2.5; 95%CI: -2.9 to -2.0).

Conclusion: Whilst systemic sclerosis-related mortality rates in adults declined from 1999 to 2023, disparities amid demographic and regional stratifications were observed; this highlights the need to select prevention and assistance programs for at-risk communities to improve survival outcomes

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/trends-and-disparities-in-systemic-sclerosis-related-mortality-rates-in-the-united-states-from-1999-to-2023/