PT  - JOURNAL ARTICLE
AU  - Pailin Ratanawatkul
AU  - Joshua J. Solomon
AU  - Darlene Kim
AU  - Marjorie P. George
AU  - Lia R. Matarrese McGibbon
AU  - M. Kristen Demoruelle
AU  - Mehrnaz Maleki-Fischbach
AU  - Isabelle Amigues
AU  - Liudmila Kastsianok
AU  - Evans R. Fernández Pérez
TI  - Trends in systemic sclerosis and systemic sclerosis-related pulmonary arterial hypertension mortality in the USA
AID  - 10.1183/23120541.00309-2019
DP  - 2020 Apr 01
TA  - ERJ Open Research
PG  - 00309-2019
VI  - 6
IP  - 2
4099  - http://openres.ersjournals.com/content/6/2/00309-2019.short
4100  - http://openres.ersjournals.com/content/6/2/00309-2019.full
SO  - erjor2020 Apr 01; 6
AB  - There are limited data nationwide on the burden of systemic sclerosis (SSc)-related mortality. We aimed to determine recent trends in SSc and SSc-related pulmonary arterial hypertension (PAH) mortality overall and across population subgroups.Using death certificate data from the National Center for Health Statistics, we computed the age-adjusted mortality rates of SSc and SSc-SSc−PAH, a lethal prevailing complication, across demographic groups, geographic regions and comorbid cardiorespiratory conditions, and used Joinpoint regression analysis to calculate the average annual percentage change (APC) in mortality.From 2003 to 2016, 25 175 death records contained a code for SSc. Decedents were predominantly female (81%) and white (73%), with an average age of 66±14 years. The age-adjusted mortality rate decreased by 3% per year from 6.6 in 2003 to 4.3 per 1 000 000 population in 2016. Also, a decreasing trend was found when SSc was stratified by age, sex, race and geographic region. The prevalence of PAH was 23%. The odds of PAH were highest in female and black decedents, and in decedents with concomitant pulmonary embolism, cardiomyopathy and interstitial lung disease (ILD). SSc−PAH mortality remained stable from 2003 to 2008 then decreased by 3% per year from 2008 to 2016. In decedents with SSc−PAH, among all concomitant comorbidities, the mortality rate associated with ILD had the highest increase (average APC 6%, 95% CI 2%−10%).The mortality rate from SSc decreased from 2003 to 2016. Decreases in mortality rates were similar across demographic groups and geographic regions. SSc−PAH-related mortality remained stable. The death rate for SSc−ILD and concomitant PAH increased during this period.While SSc-PAH-related mortality remained stable, SSc-ILD and concomitant PAH mortality increased from 2003 to 2016 http://bit.ly/3d9G0pp