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Multicenter Study
. 2011 Oct;38(10):2172-9.
doi: 10.3899/jrheum.101243. Epub 2011 Aug 15.

Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population

Monique Hinchcliff  1 , Aryeh Fischer, Elena Schiopu, Virginia D Steen; PHAROS Investigators
Affiliations
Multicenter Study

Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population

Monique Hinchcliff et al. J Rheumatol. 2011 Oct.

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.

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Figures

Figure 1
Figure 1
Flow-chart of PHAROS patients. Of the 237 PHAROS patients enrolled since study inception, 166 are classified as pre-pulmonary artery hypertension (Pre-PAH) and 71 have right-heart catheterization (RHC) confirmed incident pulmonary hypertension (PH). Sixteen patients underwent RHC for suspected PH and mean pulmonary artery pressure was normal (<25 mm Hg). Of the 71 patients with Definite PH, 49, 7 and 15 had Groups 1–3 PH respectively(32). Diastolic dysfunction=DD and interstitial lung disease=ILD.
Figure 2
Figure 2
Pie charts depicting the breakdown of patients meeting PHAROS eligibility criteria.
Figure 2
Figure 2
Pie charts depicting the breakdown of patients meeting PHAROS eligibility criteria.
See this image and copyright information in PMC

Comment in

  • The Wisdom of the PHAROS.
    Mathai SC. Mathai SC. J Rheumatol. 2011 Oct;38(10):2089-91. doi: 10.3899/jrheum.111008. J Rheumatol. 2011. PMID: 21965691 No abstract available.

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