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. 2020 Jun 15;6(2):00309-2019. doi: 10.1183/23120541.00309-2019

TABLE 2.

Average annual percentage change of age-adjusted mortality rates from systemic sclerosis and systemic sclerosisrelated pulmonary hypertension, 20032016

Variable Systemic sclerosis average annual change % (95% CI) Systemic sclerosis−pulmonary hypertension average annual change % (95% CI)
Total −3.0 (−3.3 to −2.7)# 0.1 (−2.3 to 2.6)
Sex
 Male −3.5 (−4.1 to −2.8) −1.4 (−3.0 to 0.1)
 Female −2.7 (−3.0 to −2.4) 0.5 (−2.1 to 3.2)
Race
 White −2.9 (−3.3 to −2.5) 0.1 (−2.6 to 2.9)
 Black −3.6 (−4.7 to −2.5) −0.4 (−3.1 to 2.4)
 Hispanic −3.5 (−4.7 to −2.2) −0.9 (−3.1 to 1.3)
Race/sex
 White men −3.4 (−4.2 to −2.5) −1.5 (−3.6 to 0.6)
 Black men −4.1 (−6.3 to −1.9) −1.2 (−5.6 to 3.4)
 Hispanic men −4.1 (−6.9 to −1.1) −1.5 (−12.2 to 10.3)
 White women −2.5 (−2.9 to −2.1) 0.7 (−2.0 to 3.4)
 Black women −3.4 (−4.9 to −1.9) −0.2 (−3.3 to 3.0)
 Hispanic women −3.2 (−4.3 to −2.0) −0.9 (−3.0 to 1.1)
Region
 Northeast −1.9 (−3.5 to −0.3) −0.2 (−3.6 to 3.2)
 South −4.2 (−4.9 to −3.5) −1.0 (−3.4 to 1.3)
 Midwest −2.8 (−3.9 to −1.6) 1.0 (−1.8 to 3.9)
 West −2.6 (−3.9 to −1.2) −0.9 (−3.4 to 1.7)
Age-groups years
 <35 −0.8 (−3.4 to 1.8) −1.4 (−7.1 to 4.7)
 35–54 −3.5 (−4.2 to −2.9) −1.7 (−4.6 to 1.4)
 55–74 −3.7 (−4.2 to −3.3) −1.2 (−2.7 to 0.3)
 ≥75 −1.1 (−2.7 to 0.6) 3.6 (0.9 to 6.3)
Contributing causes of death
 Respiratory
  Interstitial lung disease 1.2 (−0.2 to 2.6) 6.0 (2.0 to 10.2)
  COPD −1.7 (−3.4 to 0) 2.5 (−2.4 to 7.7)
  Pulmonary embolism −0.2 (−2.9 to 2.6) −1.5 (−5.5 to 2.7)
 Cardiovascular
  Ischaemic heart disease −4.9 (−5.7 to −4.1) 0.2 (−2.9 to 3.4)
  Cardiomyopathy −1.7 (−2.7 to −0.8) 2.7 (1.4 to 4.1)
  Hypertension −2.2 (−3.3 to −1.2) 3.1 (0.6 to 5.7)
  Valvular heart disease −2.8 (−5.8 to 0.3) 3.6 (−1.9 to 9.4)

COPD: chronic obstructive pulmonary disease. #: no joinpoint identified.; : one joinpoint identified at 2008: 2003–2008, APC 5.2% (95% CI −0.8 to 11.5); 2008–2016, APC −3.0% (−5.6 to −0.2%).