Association Between Initial Oral Therapy and Outcomes in Systemic Sclerosis-Related Pulmonary Arterial Hypertension.
Association Between Initial Oral Therapy and Outcomes in Systemic Sclerosis-Related Pulmonary Arterial Hypertension.
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DOI：
10.1002/art.39478
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发表时间：
2016-03
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通讯作者：
Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Investigators
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作者：
Lammi MR;Mathai SC;Saketkoo LA;Domsic RT;Bojanowski C;Furst DE;Steen VD;Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Investigators
比较系统性硬化症（SSc）合并肺动脉高压（PAH）患者初始口服PAH治疗的临床恶化时间（TTCW）。使用PHAROS登记处（一项纳入SSc并发肺动脉高压患者的多中心前瞻性观察研究）的数据，纳入I组PAH患者，初始治疗为内皮素受体拮抗剂（ERA）、磷酸二酯酶-5抑制剂（PDE5i）或ERA/PDE5i联合治疗6个月。主要结局为TTCW，定义为首次死亡、pah相关住院、肺移植、开始肠外注射前列环素或症状恶化。纳入98例患者（初始ERA=24，初始PDE5i=59，初始ERA/PDE5i=15）；基线变量无显著差异。与PDE5i或ERA/PDE5i相比，最初开始使用ERA的患者的TTCW明显更差（p=0.0001）。3年观察期内，ERA组死亡10例（41.6%），PDE5i组死亡4例（6.8%），ERA/PDE5i联合组死亡1例（6.7%）（p=0.004）。与较短TTCW独立相关的基线因素是初始ERA （HR 2.63, p=0.009）、较低DLCO （HR 0.69 / 10%变化，p=0.04）和较高PVR （HR 1.10 / Wood单位变化，p=0.007）。与PDE5i或ERA/PDE5i联合治疗相比，SSc-PAH患者的初始ERA治疗与TTCW显著恶化相关，即使在对普遍接受的预后因素进行调整后也是如此。需要进一步研究SSc-PAH患者的最佳初始口服治疗。
To compare time to clinical worsening (TTCW) based on initial oral PAH therapy in systemic sclerosis (SSc) patients with pulmonary arterial hypertension (PAH). Using data from the PHAROS registry (a multicenter prospective observational study enrolling SSc patients with incident pulmonary hypertension), patients with group I PAH and 6 months of initial therapy with an endothelin-receptor antagonist (ERA), phosphodiesterase-5 inhibitor (PDE5i), or a combination of ERA/PDE5i were included. The main outcome was TTCW, defined as the first occurrence of death, PAH-related hospitalization, lung transplant, initiation of parenteral prostacyclin, or worsening symptoms. Ninety-eight patients (initial ERA=24, initial PDE5i=59, initial ERA/PDE5i=15) were included; no significant differences in baseline variables existed. TTCW was significantly worse in patients initially started on ERA compared to PDE5i or ERA/PDE5i (p=0.0001). Ten patients (41.6%) in the ERA group died over the 3 year observation period, compared to 4 (6.8%) in the PDE5i group and 1 (6.7%) in the combo ERA/PDE5i group (p=0.004). Baseline factors independently associated with shorter TTCW were initial ERA (HR 2.63, p=0.009), lower DLCO (HR 0.69 per 10% change, p=0.04), and higher PVR (HR 1.10 per Wood unit change, p=0.007). Compared to PDE5i or combination ERA/PDE5i, initial therapy with an ERA in SSc-PAH patients was associated with a significantly worse TTCW, even after adjustment for commonly accepted prognostic factors. Further study into the optimal initial oral therapy in patients with SSc-PAH is needed.