Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL Identifying Systemic Sclerosis as a Unique Phenotype
Characterization of Connective Tissue Disease-Associated Pulmonary Arterial Hypertension From REVEAL Identifying Systemic Sclerosis as a Unique Phenotype
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DOI：
10.1378/chest.10-0260
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发表时间：
2010-12-01
期刊：
影响因子：
9.6
通讯作者：
Zamanian, Roham T.
中科院分区：
文献类型：
--
作者：
Chung, Lorinda;Liu, Juliana;Zamanian, Roham T.
背景 REVEAL（评估早期和长期肺动脉高压疾病管理登记处）是美国最大的经右心导管检查 (RHC) 确诊的肺动脉高压 (PAH) 患者队列，提供了比以前更全面的亚组特征。我们使用 REVEAL 来分析结缔组织病相关 PAH (CTD-APAH) 患者的临床特征。方法所有新诊断和既往诊断的世界卫生组织 (WHO) 第 1 组患者连续纳入 54 个美国中心符合 RHC 标准的 PAH 从入组时起进行横断面分析和 1 年死亡率及住院治疗分析，将 CTD-APAH 与特发性疾病、系统性硬化症 (SSc) 与系统性红斑狼疮 (SLE)、混合结缔组织病 (MCTD) 和类风湿性关节炎 (RA) 进行比较 结果 与特发性疾病患者 (n = 1,251) 相比，患有CTD-APAH (n = 641) 具有更好的血流动力学和良好的右心室超声心动图检查结果，但心包积液发生率较高，6 分钟步行距离较短（300 5 +/- 118 0 vs 329 4 +/- 134 7 m，P = 01），B 型利钠肽 (BNP) 水平较高（432 8 +/- 789 1 vs 245 6 +/- 427 2 pg/mL，P < 0001)，一氧化碳 (DLCO) 扩散能力较低（44 9% +/- 18 0% vs 63 6% +/- 22 1% 预测，P < 0001） CTD-APAH 组的一年生存率和免住院率较低（86% vs 93%，P < 0001, 67% vs 73%, P = 03) 与 SSc-APAH 患者 (n = 399) 相比，患有其他 CTD 的患者 (SLE, n = 110, MCTD, n = 52, RA, n = 28) 具有相似的血流动力学，然而，SSc-APAH 患者的 BNP 水平最高 (552 2 +/- 977 8 pg/mL)、最低 DLCO（41 2% +/- 16 3% 预测）和最差的 1 年生存率（SLE-APAH 为 82% vs 94%，MCTD-APAH 为 88%，RA-APAH 为 96%） CTD-APAH 亚组 试验注册处 ClinicalTrials gov，编号 NCT00370214，URL 临床试验 org CHEST 2010, 138(6) 1383-1394
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