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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

2013; Wiley; Volume: 66; Issue: 3 Linguagem: Inglês

10.1002/acr.22121

2151-4658

Leland W.K. Chung, Robyn T. Domsic, Bharathi Lingala, Firas Alkassab, Marcy B. Bolster, Mary Ellen Csuka, Chris T. Derk, Aryeh Fischer, Tracy Frech, Daniel E. Furst, Mardi Gomberg‐Maitland, Monique Hinchcliff, Vivien Hsu, Laura K. Hummers, Dinesh Khanna, Thomas A. Medsger, Jerry A. Molitor, Ioana R. Preston, Elena Schiopu, Lee Shapiro, Richard M. Silver, Robert W. Simms, John Varga, Jessica Gordon, Virginia Steen,

Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

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 (DL co ) <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 DL co and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.