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.
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.
Authors: Reda E Girgis; Stephen C Mathai; Jerry A Krishnan; Fredrick M Wigley; Paul M Hassoun Journal: J Heart Lung Transplant Date: 2005-10 Impact factor: 10.247
Authors: S Miyamoto; N Nagaya; T Satoh; S Kyotani; F Sakamaki; M Fujita; N Nakanishi; K Miyatake Journal: Am J Respir Crit Care Med Date: 2000-02 Impact factor: 21.405
Authors: Ronald J Raymond; Alan L Hinderliter; Park W Willis; David Ralph; Edgar J Caldwell; William Williams; Neil A Ettinger; Nicholas S Hill; Warren R Summer; Bennett de Boisblanc; Todd Schwartz; Gary Koch; Linda M Clayton; Maria M Jöbsis; James W Crow; Walker Long Journal: J Am Coll Cardiol Date: 2002-04-03 Impact factor: 24.094
Authors: Steven M Kawut; Darren B Taichman; Christine L Archer-Chicko; Harold I Palevsky; Stephen E Kimmel Journal: Chest Date: 2003-02 Impact factor: 9.410
Authors: D Mukerjee; D St George; B Coleiro; C Knight; C P Denton; J Davar; C M Black; J G Coghlan Journal: Ann Rheum Dis Date: 2003-11 Impact factor: 19.103
Authors: Matthew R Lammi; Stephen C Mathai; Lesley Ann Saketkoo; Robyn T Domsic; Christine Bojanowski; Daniel E Furst; Virginia D Steen Journal: Arthritis Rheumatol Date: 2016-03 Impact factor: 10.995
Authors: Mei-Sing Ong; Steve Abman; Eric D Austin; Jeffrey A Feinstein; Rachel K Hopper; Usha S Krishnan; Mary P Mullen; Marc D Natter; J Usha Raj; Erika B Rosenzweig; Kenneth D Mandl Journal: J Pediatr Date: 2019-06-05 Impact factor: 4.406