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结节病相关性肺动脉高压。

Sarcoidosis-Associated Pulmonary Hypertension.

机构信息

Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands.

Department of Pulmonology, St. Antonius Hospital, Nieuwegein, The Netherlands.

出版信息

Semin Respir Crit Care Med. 2020 Oct;41(5):659-672. doi: 10.1055/s-0040-1713615. Epub 2020 Aug 10.

Abstract

Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of ≥25 mm Hg. Since both PH and sarcoidosis are rare diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred to a tertiary center up to 79% in patients awaiting lung transplant. Most patients with SAPH show advanced parenchymal disease as the underlying mechanism. However, some patients have disproportional elevated pulmonary artery pressure, and PH can occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be raised based on symptoms or tests, such as pulmonary function tests, laboratory findings, electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography. Right heart catheterization should be considered on a case-by-case basis in patients with clinical suspicion of PH, taking into account clinical consequences. Treatment options are considered on patient level in a PH expert center, and might include oxygen therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence is scarce. Furthermore, in a subset of patients, interventional therapy or eventually lung transplant can be considered. SAPH is associated with high morbidity. Mortality is higher in sarcoidosis patients with PH compared with those without PH, and increases in patients with more advanced stages of sarcoidosis and/or PH.

摘要

肺动脉高压(PH)是结节病的一种已知并发症,定义为平均肺动脉压≥25mmHg。由于 PH 和结节病都是罕见疾病,因此 SAPH 的数据主要来自小型回顾性研究。SAPH 的估计患病率在转诊至三级中心的患者中为 3%,在等待肺移植的患者中高达 79%。SAPH 患者大多表现为间质性疾病,这是其潜在机制。然而,一些患者的肺动脉压升高不成比例,且 SAPH 可发生于无间质性疾病的结节病患者中。其他机制如血管疾病、肺栓塞、毛细血管后 PH、外在压迫和其他结节病相关的合并症可能导致 SAPH。由于结节病的症状和体征重叠,因此诊断 PH 具有挑战性。可以根据症状或检查(如肺功能检查、实验室发现、心电图或胸部 CT)来怀疑 PH。PH 筛查主要依赖于经胸超声心动图。对于有 PH 临床怀疑的患者,应根据具体情况考虑进行右心导管检查,同时考虑临床后果。在 PH 专家中心,根据患者个体情况考虑治疗方案,可能包括氧疗、免疫抑制或 PH 特异性治疗。但是,证据质量较低。此外,在一部分患者中,可以考虑介入治疗或最终进行肺移植。SAPH 与高发病率相关。与无 PH 的结节病患者相比,有 PH 的结节病患者死亡率更高,而且在间质性疾病和/或 PH 分期更严重的患者中死亡率增加。

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