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

被引：376

作者：

Chung, Lorinda ^{[1
,2
]}

Liu, Juliana ^{[3
,8
]}

Parsons, Lori ^{[4
]}

Hassoun, Paul M. ^{[5
]}

McGoon, Michael ^{[6
]}

Badesch, David B. ^{[7
]}

Miller, Dave P. ^{[4
]}

Nicolls, Mark R. ^{[1
,3
,8
]}

Zamanian, Roham T. ^{[3
,8
]}

机构：

[1] VA Palo Alto Hlth Care Syst, Palo Alto, CA 94304 USA

[2] Stanford Univ, Div Rheumatol & Immunol, Stanford, CA 94305 USA

[3] Stanford Univ, Div Pulm & Crit Care Med, Stanford, CA 94305 USA

[4] ICON Clin Res, San Francisco, CA USA

[5] Johns Hopkins Univ, Div Pulm & Crit Care Med, Baltimore, MD USA

[6] Mayo Clin, Div Cardiol, Rochester, MN USA

[7] Univ Colorado, Div Pulm & Crit Care Med, Denver, CO 80202 USA

[8] Vera Moulton Wall Ctr Pulm Vasc Dis, Stanford, CA USA

来源：

CHEST | 2010年 / 138卷 / 06期

关键词：

BRAIN NATRIURETIC PEPTIDE; IMMUNOSUPPRESSIVE THERAPY; SCLERODERMA SPECTRUM; DIFFUSING-CAPACITY; BOSENTAN TREATMENT; CARBON-MONOXIDE; PREDICTORS; SURVIVAL; PREVALENCE; ANTIBODIES;

D O I：

10.1378/chest.10-0260

中图分类号：

R4 [临床医学];

学科分类号：

100218 [急诊医学];

摘要：

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 org CHEST 2010, 138(6) 1383-1394

引用

页码：1383 / 1394

页数：12

共 39 条

[1]

High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis [J].