Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): Baseline Characteristics and Description of Study Population
- 15 August 2011
- journal article
- research article
- Published by The Journal of Rheumatology in The Journal of Rheumatology
- Vol. 38 (10), 2172-2179
- https://doi.org/10.3899/jrheum.101243
Abstract
Objective.Pulmonary arterial hypertension (PAH) increases mortality in systemic sclerosis (SSc). The multicenter PHAROS registry (Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma) prospectively follows subjects with SSc at high risk for or with incident pulmonary hypertension (PH). We describe the registry design and baseline characteristics of subjects enrolled during the first 18 months since the start of the study.Methods.High-risk subjects are enrolled and classified as Pre-PAH if they have (1) carbon monoxide diffusing capacity (DLCO) < 55% predicted; (2) percentage of predicted forced vital capacity/DLCO ratio ≥ 1.6; or (3) an estimated right ventricular systolic pressure > 35 mm Hg on echocardiography. Subjects with right heart catheterization (RHC)-confirmed incident PH (mean pulmonary artery pressure ≥ 25 mm Hg within previous 6 months) are subclassified into PAH, pulmonary venous hypertension secondary to left-side heart disease (PVH), and PH due to interstitial lung disease (PH-ILD). Baseline and biannual demographic, clinical, and laboratory data and patient-reported health questionnaires are collected.Results.There are 237 subjects enrolled in PHAROS. The majority are white (73%) and women (87%). There are 166 Pre-PAH and 71 Definite PH subjects (49 PAH, 7 PVH, and 15 PH-ILD).Conclusion.PHAROS is the largest US and Canadian cohort of subjects with SSc at high risk for or with incident PAH. PAH-specific therapies are approved for 49/71 subjects with RHC-confirmed PAH. Analyses of PHAROS registry data will permit identification of risk factors for development of PAH among SSc patients at high risk for PAH and enhance understanding of the course of SSc-PAH.Keywords
This publication has 32 references indexed in Scilit:
- Pulmonary hypertension and pulmonary arterial hypertension: a clarification is neededEuropean Respiratory Journal, 2010
- Diagnosis and Assessment of Pulmonary Arterial HypertensionJournal of the American College of Cardiology, 2009
- Tadalafil Therapy for Pulmonary Arterial HypertensionCirculation, 2009
- EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)Annals Of The Rheumatic Diseases, 2009
- The Heart in SclerodermaRheumatic Disease Clinics of North America, 2008
- Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseasesAnnals Of The Rheumatic Diseases, 2007
- Long-Term Outcome of Bosentan Treatment in Idiopathic Pulmonary Arterial Hypertension and Pulmonary Arterial Hypertension Associated with the Scleroderma Spectrum of DiseasesThe Journal of Heart and Lung Transplantation, 2005
- Clinical classification of pulmonary hypertensionJournal of the American College of Cardiology, 2004
- The MOS 36-ltem Short-Form Health Survey (SF-36)Medical Care, 1992
- Preliminary criteria for the classification of systemic sclerosis (scleroderma)Arthritis & Rheumatism, 1980