Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
BMJ Open. 2013 Aug 1;3(8):e003113. doi: 10.1136/bmjopen-2013-003113.
Few studies have focused on pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTDs). The optimal treatment for CTD-PAH has yet to be established.
Meta-analysis of the data from evaluations of treatment for PAH generally (19 studies) and CTD-PAH specifically (nine studies) to compare the effects of pulmonary vasodilative PAH agents. MEDLINE, EMBASE and BIOSIS were searched. English-language full-text articles published between January 1990 and August 2012 were eligible.
International.
Patients with PAH generally (n=3073) and CTD-PAH specifically (n=678).
Exercise capacity (6 min walk distance, 6 MWD).
Patients with PAH (all forms) had mean age 32-55 years (women, 61-87%); CTD-PAH patients had mean age 45-55 years (women, 74-95%). Overall estimate of mean change in 6 MWD from baseline (95% CI) for the active treatment group versus the control group in all patients with PAH was 34.6 m (27.4-41.9 m). Pooled mean differences from the results for patients receiving placebo by subgroup of patients receiving phosphodiesterase (PDE)-5 inhibitors, endothelin receptor antagonists (ERAs) and prostacyclin (PGI2) analogues were 22.4-45.5, 39.5-44.2 and 12.4-64.9 m, respectively. Overall estimate of mean difference between changes in 6 MWD in patients with CTD-PAH was 34.2 m (23.3-45.0 m). Pooled mean differences by subgroup of patients receiving PDE-5 inhibitors, ERAs and PGI2 analogues in patients with CTD-PAH were 37.0-47.1, 14.1-21.7 and 21.0-108.0 m, respectively. ERAs were less effective in patients with CTD-PAH than all-form patients with PAH: 14.1 m (-4.4-32.6 m) vs 39.5 m (19.5-59.6 m) for bosentan and 21.7 m (2.2-41.3 m) vs 44.2 m (30.2-58.2 m) for ambrisentan.
All three types of PAH agent are effective. However, ERAs may be a less effective choice against CTD-PAH; further studies are needed. Limitations include the limited number of studies for some agents and for patients with CTD-PAH.
鲜有研究关注与结缔组织疾病（CTD）相关的肺动脉高压（PAH）。CTD-PAH 的最佳治疗方法尚未确定。
对一般 PAH（19 项研究）和 CTD-PAH（9 项研究）治疗评估数据的荟萃分析，以比较肺血管扩张性 PAH 药物的效果。检索 MEDLINE、EMBASE 和 BIOSIS。入选 1990 年 1 月至 2012 年 8 月发表的英文全文文章。
国际。
一般 PAH 患者（n=3073）和 CTD-PAH 患者（n=678）。
运动能力（6 分钟步行距离，6MWD）。
PAH 患者（所有类型）的平均年龄为 32-55 岁（女性，61-87%）；CTD-PAH 患者的平均年龄为 45-55 岁（女性，74-95%）。总体估计所有 PAH 患者中，活性治疗组与对照组之间的 6MWD 从基线的平均变化（95%CI）为 34.6m（27.4-41.9m）。根据接受磷酸二酯酶（PDE）-5 抑制剂、内皮素受体拮抗剂（ERA）和前列环素（PGI2）类似物的患者亚组的结果，接受安慰剂的患者的平均差异分别为 22.4-45.5、39.5-44.2 和 12.4-64.9m。CTD-PAH 患者的 6MWD 变化之间的平均差异估计为 34.2m（23.3-45.0m）。根据接受 PDE-5 抑制剂、ERA 和 PGI2 类似物的患者亚组，CTD-PAH 患者的平均差异分别为 37.0-47.1、14.1-21.7 和 21.0-108.0m。与所有形式的 PAH 患者相比，ERA 在 CTD-PAH 患者中的疗效较低：波生坦为 14.1m（-4.4-32.6m），安贝生坦为 44.2m（30.2-58.2m）；替地沙坦为 21.7m（2.2-41.3m），安立生坦为 21.0-108.0m。
所有三种类型的 PAH 药物均有效。然而，ERA 可能是治疗 CTD-PAH 的较差选择；需要进一步研究。限制包括某些药物和 CTD-PAH 患者的研究数量有限。
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