Department of Medicine, University of South Florida.
Interstitial Lung Disease and Sarcoidosis Program, Center for Advanced Lung Disease and Lung Transplant, Tampa General Hospital, Tampa, Florida, USA.
Curr Opin Pulm Med. 2020 Sep;26(5):582-590. doi: 10.1097/MCP.0000000000000701.
Pulmonary hypertension in sarcoidosis is a well known entity. Sarcoidosis-associated pulmonary hypertension (SAPH) incurs substantial morbidity and mortality. This review examines recent literatures published on epidemiology, prognosis and therapeutic management in SAPH.
Several registries have been published between 2017 and 2020. The consensus conclusion - SAPH is a harbinger for poor prognosis. Several factors were noted for predicting adverse outcome in SAPH like reduced 6-min walk distance and diffusing capacity for carbon monoxide. Given its adverse outcome, experts have now focused on methods for early screening of SAPH in sarcoid patients. The exploration of pulmonary vasodilator drugs in SAPH is ongoing. In recent times, trials have been published utilizing Macitentan and parenteral prostacyclin in severe SAPH. Although these trials show encouraging results, the evidence from these studies are limited to approve these agents as preferred drugs for treating SAPH. A large multicentric trial of drugs used for pulmonary arterial hypertension with meaningful, yet feasible, event driven endpoint is still lacking. Lately, interventional treatment by pulmonary artery balloon pulmonary angioplasty and stenting has gained traction for treating pulmonary artery stenosis and chronic thromboembolic pulmonary hypertension. However, the conclusion is still based on small cohorts or case series.
Several registries have highlighted SAPH portends an unfavorable consequence. On the contrary, no published guideline exists to treat SAPH. The precise role of immunosuppressive agents is unclear. The limited evidence favoring use of pulmonary vasodilators arise from small retrospective case series and/or single-center nonrandomized observational studies. Further multicenter randomized research is warranted to better define patient population to treat and how best to treat them.
结节病相关性肺动脉高压（SAPH）是一种明确的病症。SAPH 会导致较高的发病率和死亡率。本综述分析了 2017 年至 2020 年间发表的有关 SAPH 的流行病学、预后和治疗管理的最新文献。
2017 年至 2020 年间发表了几项注册研究。专家们一致认为 SAPH 是预后不良的标志。有几个因素被认为可以预测 SAPH 的不良预后，例如 6 分钟步行距离和一氧化碳弥散量的降低。鉴于其不良预后，专家们现在专注于寻找在结节病患者中筛查 SAPH 的早期方法。对 SAPH 中肺动脉扩张剂药物的探索仍在继续。最近，已经发表了利用马西替坦和前列腺素 I2 治疗严重 SAPH 的试验。尽管这些试验结果令人鼓舞，但这些研究的证据还不足以批准这些药物作为治疗 SAPH 的首选药物。一项针对肺动脉高压药物的大型多中心、具有有意义但可行的终点事件驱动的试验仍然缺乏。最近，经皮球囊肺动脉血管成形术和支架置入术的介入治疗方法已被用于治疗肺动脉狭窄和慢性血栓栓塞性肺动脉高压，得到了广泛关注。然而，目前的结论仍然基于小样本队列或病例系列。
几项注册研究强调 SAPH 预示着不良后果。相反，目前尚无治疗 SAPH 的指南。免疫抑制剂的确切作用尚不清楚。支持使用肺动脉扩张剂的有限证据来自于小型回顾性病例系列和/或单中心非随机观察性研究。需要进一步的多中心随机研究来更好地确定治疗患者人群，并确定最佳治疗方法。
