Background

Pulmonary angioplasty has been performed in patients with sarcoidosis-associated pulmonary hypertension (SAPH) but most evidence comes from case reports and small case series. Overall outcomes remain unclear. We conducted an individual participant data (IPD) meta-analysis of baseline, procedural, and outcome data of pulmonary angioplasty in patients with SAPH.

Methods

We performed searches and systematically reviewed references from PubMed, Embase, Cochrane, ClinicalTrials.gov, and grey literature. We included IPD of patients who underwent pulmonary angioplasty for SAPH. Those without definitive diagnosis of sarcoidosis or with other causes of pulmonary vascular stenosis or compression were excluded.

Results

Of 1,293 screened references, seven were included. IPD was obtained for 17 patients (median age 60 (55-65) years; 82.4% female); most of whom were Scadding stages III or IV and had NYHA FC III or IV. All patients with documented changes in six-minute-walk distance (6MWD) had a significant improvement that ranged from 12.6 to 102.4% (p < 0.01). There were no deaths during a median follow-up of 6 (IQR 3-18) months.

Conclusions

Pulmonary angioplasty with or without stenting of focal stenosis or compressions of pulmonary vessels may lead to significant improvement in 6MWD in patients with SAPH. However, this study had a small sample and some methodological limitations, such as analysis mostly of case reports and series. Randomized controlled clinical trials and/or large multicenter registry studies are needed to provide higher evidence in this topic.

中文翻译：

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结节病相关性肺动脉高压伴或不伴支架置入术的肺血管成形术的特征和结果：系统评价和个体参加者数据荟萃分析。

背景

结节病相关性肺动脉高压（SAPH）患者已进行了肺血管成形术，但大多数证据来自病例报告和小病例系列。总体结果仍不清楚。我们对SAPH患者的肺血管成形术的基线，手术和结局数据进行了单独的参与者数据（IPD）荟萃分析。

方法

我们进行了搜索并系统审查了PubMed，Embase，Cochrane，ClinicalTrials.gov和灰色文献的参考文献。我们纳入了接受SAPH肺血管成形术治疗的患者的IPD。没有明确诊断结节病或其他原因导致肺血管狭窄或压迫的患者被排除在外。

结果

在1,293篇筛选的参考文献中，包括了7篇。IPD获得了17例患者（中位年龄为60（55-65）岁；女性为82.4％）；他们中的大多数人处于第三阶段或第四阶段，并且有NYHA FC III或IV。所有记录有六分钟步行距离（6MWD）变化的患者均有明显改善，范围为12.6％至102.4％（p <0.01）。在中位随访6个月（IQR 3-18）期间没有死亡。

结论

伴有或不伴有局灶性狭窄或肺血管受压的肺血管成形术可导致SAPH患者的6MWD显着改善。但是，该研究样本少，方法学上也有局限性，例如大部分病例报告和系列分析。需要进行随机对照临床试验和/或大型多中心注册表研究，以提供有关该主题的更高证据。