TY - JOUR
T1 - Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL
T2 - Identifying systemic sclerosis as a unique phenotype
AU - Chung, Lorinda
AU - Liu, Juliana
AU - Parsons, Lori
AU - Hassoun, Paul M.
AU - McGoon, Michael
AU - Badesch, David B.
AU - Miller, Dave P.
AU - Nicolls, Mark R.
AU - Zamanian, Roham T.
N1 - Funding Information:
Role of sponsors: Wolters Kluwer has coordinated feedback among the authors; this support was funded by Actelion Pharmaceuticals US, Inc.
Funding Information:
Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Chung has received research support funding from Gilead Sciences and United Therapeutics Corp and has served on the Speakers' Bureau for Acetlion Pharmaceuticals (< $10,000 per year). Ms Liu has served as a consultant to Gilead Sciences and United Therapeutics Corp and participated in advisory board meetings for Actelion Pharmaceuticals. Ms Parsons is an employee of ICON Clinical Research, a company that receives research funding from Actelion and other pharmaceutical companies. Dr Hassoun has received research funding support from Actelion/CoTherix Inc and is on the Advisory Board for Novartis. Dr McGoon has received research funding from Gilead and Medtronic Inc. He has served on steering committees for Gilead and Lung Rx Inc and participated on clinical end point committees in studies sponsored by Actelion. He is on a Data Safety Monitoring Board for a study sponsored by Gilead. Dr McGoon has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Badesch has received honoraria for service on Steering Committees and/or Advisory Boards for Actelion/CoTherix, Gilead/Myogen Inc Encysive Pharmaceuticals, Pfizer, GlaxoSmithKline, Lung Rx Inc, United Therapeutics, Eli Lilly and Company/ICOS, Biogen Idec Inc, mondoBIOTECH AG/mondoGEN AG, and Bayer. Dr Badesch has received grants from Actelion/CoTherix, Gilead/Myogen, Encysive Pharmaceuticals, Pfizer, United Therapeutics, Lung Rx Inc, Eli Lilly and Company/ICOS, Bayer, Novartis, and NIH/NHLBI. Dr Badesch has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Mr Miller is an employee of ICON Clinical Research, a company that receives research funding from Actelion and other pharmaceutical companies. Dr Nicolls has reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article. Dr Zamanian has received research funding support through the Entelligence-Actelion career development research grant ($75,000) and has served as a consultant to United Therapeutics and Gilead pharmaceuticals.
Funding Information:
Funding/Support: The REVEAL Registry was sponsored by Actelion Pharmaceuticals US, Inc. Dr Chung receives funding support from the Scleroderma Research Foundation.
PY - 2010/12/1
Y1 - 2010/12/1
N2 - 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.
AB - 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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U2 - 10.1378/chest.10-0260
DO - 10.1378/chest.10-0260
M3 - Article
C2 - 20507945
AN - SCOPUS:78649315195
VL - 138
SP - 1383
EP - 1394
JO - Diseases of the chest
JF - Diseases of the chest
SN - 0012-3692
IS - 6
ER -