Background/Purpose: A comprehensive evaluation of long-term trends in systemic sclerosis (SSc) mortality is important to understand the influence of recent advances in SSc management and to identify groups at the highest risk of death. Our objective is to identify secular trends in SSc mortality, and trends in SSc mortality relative to mortality from all other causes (non-SSc).

Methods: We used national mortality and census database to calculate age-standardized mortality rate (ASMR) for SSc and non-SSc causes, ratio of SSc-ASMR to non-SSc-ASMR, and proportions of SSc and non-SSc deaths by age groups for each year from 1968 through 2015. We performed joinpoint trend analysis to estimate annual percent change (APC) and average APC (AAPC) for the above measures for the total population and by sex, race, and age.

Results: From 1968 to 2015, there were 46,798 SSc deaths and 106,058,839 non-SSc deaths. The ASMR for SSc was 2.7 (95% CI, 2.4-2.9) per million persons in 1968 and 3.2 (95% CI, 3.0-3.4) per million persons in 2015. Joinpoint trend analysis showed that SSc-ASMR increased at an APC of 1.0% between 1968 and 1987, and continued to increase at a higher APC (2.2%) from 1987 to 2001, before decreasing starting in 2001 (APC, -2.6%; 95% CI, -2.2% to -3.1%; 2001-2015). In contrast to this rise-and-decline trend in SSc-ASMR, the non-SSc-ASMR continuously decreased throughout the study period. The resulting SSc-ASMR:non-SSc-ASMR ratio was 111.6% higher in 2015 than in 1968. Men with SSc died at younger ages than did women with SSc. However, women had greater annual increases in SSc-ASMR than did men from 1968 to 2000. Black persons had higher SSc-ASMRs and died at younger ages than did white persons. However, over the entire study period, the SSc-ASMR decreased in black persons (AAPC, -0.4%, 95% CI, -0.7% to -0.0%) while it increased in white persons (AAPC, 0.4%, 95% CI, 0.1% to 0.6%). Persons aged ≥65 years had the steepest increases in SSc-ASMRs (AAPC, 2.0%; 95% CI, 1.8% to 2.2%; cumulative, 187.0%), whereas those aged ≤44 years had the largest decrease (AAPC, -1.9%; 95% CI, -0.2.5% to -1.2%; cumulative -60.0%) over the 48-year period. Consistently, the proportions of SSc deaths significantly decreased in ≤44 and 45-54 year age groups, but increased at a higher APC in ≥65 year old decedents.

Conclusion: After continuously increasing for 33-years (1968-2000), SSc mortality decreased during 2001-2015. The most improvement in SSc mortality was seen in younger age groups. However, the improvement in SSc mortality has not kept up with the improvement in non-SSc mortality. Racial and sex disparities persist in SSc mortality. Men and black persons with SSc die at younger ages.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/48-year-trends-in-systemic-sclerosis-mortality-in-the-united-states-1968-2015-steady-decrease-for-15-years-after-33-years-of-continuous-increase/