Is Mortality Improving in Systemic Sclerosis? Save

Systemic sclerosis (SSc) is a progressive autoimmune disorder that causes premature death, usually from lung, GI, or renal disease. While treatment efforts have been frustrating advances in care may have altered the risk of death according to population data showing that while SSc mortality increased from 1968 through 2000, it has declined since 2001.

through 2015. However, SSc mortality relative to non‐SSc mortality remains high. SSc mortality has disproportionately changed by sex and race over the 48 years.

U.S. national mortality statistics from the Center for Disease Control and Prevention and Census Bureau were used to calculate age‐standardized mortality rate (ASMR) for SSc and non‐SSc (all other causes).

Between 1968 and 2015, there were 46,798 deaths in SSc compared to 106,058,839 non‐SSc deaths.

Over this 48 year period, the mortality rate (ASMR) decreased in the non‐SSc cohort.  Yet, between 1968‐2000 the ASMR increased for SSc patients at an annual percentage rate of 1-2.2%.  But since 2001, SSc ASMR decreases each year. Nevertheless, the ASMR remains elevated at 3.2 per million - which is 18.5% higher than that seen in 1968 and has risen while the ASMR has dropped in the non-SSc population. 

Improved survival rates in SSC could be from more treatment options, better management of complications, newer classification criteria facilitating diagnosis and possibly the use of less toxic medications.