从 REVEAL 研究中对结缔组织病相关肺动脉高压的特征分析：确定系统性硬化症为一种独特表型。
Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype.
机构信息
VA Palo Alto Health Care System, Palo Alto, CA 94304, USA.
出版信息
Chest. 2010 Dec;138(6):1383-94. doi: 10.1378/chest.10-0260. Epub 2010 May 27.
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.
背景
REVEAL（评估早期和长期肺动脉高压疾病管理的登记处）是美国最大的一组经右心导管检查（RHC）确诊的肺动脉高压（PAH）患者队列，比以前可能的情况提供了更全面的亚组特征描述。我们使用 REVEAL 分析了结缔组织病相关 PAH（CTD-APAH）患者的临床特征。
方法
所有在 54 个美国中心符合世界卫生组织（WHO）第 1 组 PAH 标准并进行 RHC 的新诊断和既往诊断患者均连续入组。通过从入组时开始的横断面和 1 年死亡率和住院率分析，将 CTD-APAH 与特发性疾病和系统性硬化症（SSc）与系统性红斑狼疮（SLE）、混合性结缔组织病（MCTD）和类风湿关节炎（RA）进行比较。
结果
与特发性疾病患者（n=1251）相比，CTD-APAH 患者（n=641）的血流动力学更好，右心室超声心动图检查结果有利，但心包积液的发生率更高，6 分钟步行距离更短（300.5±118.0 vs. 329.4±134.7 m，P=0.01），B 型利钠肽（BNP）水平更高（432.8±789.1 vs. 245.6±427.2 pg/ml，P<0.0001），一氧化碳弥散量（Dlco）更低（44.9%±18.0%预测值 vs. 63.6%±22.1%预测值，P<0.0001）。CTD-APAH 组 1 年生存率和免于住院率较低（86% vs. 93%，P<0.0001；67% vs. 73%，P=0.03）。与 SSc-APAH 患者（n=399）相比，其他 CTD 患者（SLE，n=110；MCTD，n=52；RA，n=28）的血流动力学相似；然而，SSc-APAH 患者的 BNP 水平最高（552.2±977.8 pg/ml），Dlco 最低（41.2%±16.3%预测值），1 年生存率最差（SLE-APAH 为 82%，MCTD-APAH 为 88%，RA-APAH 为 96%）。
结论
SSc-APAH 患者表现出独特的表型，所有 CTD-APAH 亚组中 BNP 水平最高，Dlco 最低，生存率最差。
试验注册
ClinicalTrials.gov；编号：NCT00370214；网址：clinicaltrials.gov。
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