Long-Term Outcomes in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS)

Chest
Kathleen D KolstadPHAROS Investigators

Abstract

Pulmonary arterial hypertension (PAH) is a leading cause of death in patients with systemic sclerosis (SSc). The purpose of this study was to assess long-term outcomes in patients with SSc-PAH. Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma is a prospective registry of patients with SSc at high risk for or with incident pulmonary hypertension from right heart catheterization. Incident World Health Organization group I PAH patients were analyzed. Kaplan-Meier survival curves were generated for the overall cohort and those who died of PAH. Multivariate Cox regression models identified predictors of mortality. Survival in 160 patients with incident SSc-PAH at 1, 3, 5, and 8 years was 95%, 75%, 63%, and 49%, respectively. PAH accounted for 52% of all deaths. When restricted to deaths from PAH, respective survival rates were 97%, 83%, 76%, and 76%, with 93% of PAH-related deaths occurring within 4 years of diagnosis. Men (hazard ratio [HR], 3.11; 95% CI, 1.38-6.98), diffuse disease (HR, 2.12; 95% CI, 1.13-3.93), systolic pulmonary artery pressure (PAP) on ECG (HR, 1.06 95% CI, 1.01-1.11), mean PAP on right heart catheterization (HR, 1.03; 95% CI, 1.001-1.07), 6-min walk distance (HR, 0.92; 95% CI, 0.86-...Continue Reading

Citations

Feb 7, 2019·Rheumatology International·Durga Prasanna Misra, Vikas Agarwal
May 24, 2019·Annals of the Rheumatic Diseases·Siddharth Jain, Shefali Khanna Sharma
May 28, 2019·Clinical Rheumatology·Bihter SenturkMerih Birlik
Aug 2, 2019·European Respiratory Review : an Official Journal of the European Respiratory Society·Jason WeatheraldMarc Humbert
Jun 25, 2020·ERJ Open Research·Pailin RatanawatkulEvans R Fernández Pérez
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Jun 17, 2020·European Journal of Internal Medicine·Cosimo BruniMarco Matucci-Cerinic
Mar 12, 2021·British Journal of Pharmacology·Pritesh P JainJason X-J Yuan

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