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Original Research: PULMONARY ARTERIAL HYPERTENSION |

Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL: Identifying Systemic Sclerosis as a Unique Phenotype

Lorinda Chung, MD; Juliana Liu, MSN; Lori Parsons, BS; Paul M. Hassoun, MD, FCCP; Michael McGoon, MD, FCCP; David B. Badesch, MD; Dave P. Miller, MS; Mark R. Nicolls, MD; Roham T. Zamanian, MD
Author and Funding Information

From the Division of Immunology and Rheumatology (Dr Chung), and the Division of Pulmonary and Critical Care Medicine (Ms Liu and Drs Nicolls and Zamanian), Stanford University, Stanford, CA; the Veteran Affairs Palo Alto Health Care System (Drs Chung and Nicolls), Palo Alto, CA; ICON Clinical Research (Ms Parsons and Mr Miller), San Francisco, CA; the Division of Pulmonary and Critical Care Medicine (Dr Hassoun), Johns Hopkins University, Baltimore, MD; the Division of Cardiology (Dr McGoon), Mayo Clinic, Rochester, MN; the Division of Pulmonary and Critical Care Medicine (Dr Badesch), University of Colorado, Denver, CO; and the Vera Moulton Wall Center for Pulmonary Vascular Disease (Ms Liu and Drs Nicolls and Zamanian), Stanford, CA.

Correspondence to: Lorinda Chung, MD, 3801 Miranda Ave, VA Palo Alto Health Care System, Palo Alto, CA 94304; e-mail: shauwei@stanford.edu


Funding/Support: The REVEAL Registry was sponsored by Actelion Pharmaceuticals US, Inc. Dr Chung receives funding support from the Scleroderma Research Foundation.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2010 American College of Chest Physicians


Chest. 2010;138(6):1383-1394. doi:10.1378/chest.10-0260
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Background:  REVEAL (the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) is the largest US cohort of patients with pulmonary arterial hypertension (PAH) confirmed by right-sided heart catheterization (RHC), providing a more comprehensive subgroup characterization than previously possible. We used REVEAL to analyze the clinical features of patients with connective tissue disease-associated PAH (CTD-APAH).

Methods:  All newly and previously diagnosed patients with World Health Organization (WHO) group 1 PAH meeting RHC criteria at 54 US centers were consecutively enrolled. Cross-sectional and 1-year mortality and hospitalization analyses from time of enrollment compared CTD-APAH to idiopathic disease and systemic sclerosis (SSc) to systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), and rheumatoid arthritis (RA).

Results:  Compared with patients with idiopathic disease (n = 1,251), patients with CTD-APAH (n = 641) had better hemodynamics and favorable right ventricular echocardiographic findings but a higher prevalence of pericardial effusions, lower 6-min walk distance (300.5 ± 118.0 vs 329.4 ± 134.7 m, P = .01), higher B-type natriuretic peptide (BNP) levels (432.8 ± 789.1 vs 245.6 ± 427.2 pg/mL, P < .0001), and lower diffusing capacity of carbon monoxide (Dlco) (44.9% ± 18.0% vs 63.6% ± 22.1% predicted, P < .0001). One-year survival and freedom from hospitalization were lower in the CTD-APAH group (86% vs 93%, P < .0001; 67% vs 73%, P = .03). Compared with patients with SSc-APAH (n = 399), those with other CTDs (SLE, n = 110; MCTD, n = 52; RA, n = 28) had similar hemodynamics; however, patients with SSc-APAH had the highest BNP levels (552.2 ± 977.8 pg/mL), lowest Dlco (41.2% ± 16.3% predicted), and poorest 1-year survival (82% vs 94% in SLE-APAH, 88% in MCTD-APAH, and 96% in RA-APAH).

Conclusions:  Patients with SSc-APAH demonstrate a unique phenotype with the highest BNP levels, lowest Dlco, and poorest survival of all CTD-APAH subgroups.

Trial registry:  ClinicalTrials.gov; No.: NCT00370214; URL: clinicaltrials.gov

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