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The impact of PAH

Among SSc decedents, the prevalence of PAH was 23% (n=5765). The odds of PAH were higher in female and black decedents, in the 55–74 age groups and decedents with pulmonary embolism, cardiomyopathy and interstitial lung disease (table 1).

Among subjects with SSc−PAH, joinpoint analysis showed that, in contrast to other respiratory or cardiovascular concomitant contributing causes-of-death, the mortality rate associated with interstitial lung disease (ILD) had the highest average annual percentage change increased during the study period (6%, 95% CI 2 to 10%).

The annual percentage change of SSc-related PAH mortality remained stable between 2003 and 2008 then decreased by 3% from 2008 to 2016. However, when the annual percentage change was averaged over the 2003–2016 period, there was no significant change (average annual percentage change 0.1%, 95% CI −2.3 to 2.6%, table 2).

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