Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry. Arthritis Care Res (Hoboken) 2014 Mar;66(3):489-95
Date
08/29/2013Pubmed ID
23983198DOI
10.1002/acr.22121Scopus ID
2-s2.0-84896692199 (requires institutional sign-in at Scopus site) 128 CitationsAbstract
OBJECTIVE: To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US.
METHODS: The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure >25 mm Hg and pulmonary capillary wedge pressure <15 mm Hg without significant interstitial lung disease) were included in these analyses.
RESULTS: In total, 131 SSc patients with incident PAH were followed for a mean ± SD of 2.0 ± 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1- 8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8 -22.8), and diffusing capacity for carbon monoxide (DLCO) <39% predicted (HR 4.2, 95% CI 1.3-13.8) were significant predictors of mortality.
CONCLUSION: This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLCO and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.
Author List
Chung L, Domsic RT, Lingala B, Alkassab F, Bolster M, Csuka ME, Derk C, Fischer A, Frech T, Furst DE, Gomberg-Maitland M, Hinchcliff M, Hsu V, Hummers LK, Khanna D, Medsger TA Jr, Molitor JA, Preston IR, Schiopu E, Shapiro L, Silver R, Simms R, Varga J, Gordon JK, Steen VDAuthor
Mary Ellen Csuka MD Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AgedFamilial Primary Pulmonary Hypertension
Female
Humans
Hypertension, Pulmonary
Longitudinal Studies
Male
Middle Aged
Prospective Studies
Registries
Scleroderma, Systemic
United States