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结节病相关性肺动脉高压:综述。

Pulmonary hypertension in sarcoidosis: a review.

机构信息

Royal Brompton Hospital and National Heart and Lung Institute Imperial College, London, UK.

出版信息

Respirology. 2011 Jan;16(1):69-77. doi: 10.1111/j.1440-1843.2010.01872.x.

Abstract

Pulmonary hypertension (PH) is a well-recognized complication of sarcoidosis. Patients with sarcoidosis-associated PH (SAPH) have poorer functional status and greater supplemental oxygen requirements than sarcoidosis patients without PH, and are more likely to be listed for lung transplantation. PH is an independent risk factor for mortality in sarcoidosis patients awaiting lung transplantation. The pathophysiology of SAPH is complex, with multiple mechanisms contributing to pathogenesis, including the fibrous destruction of the pulmonary vascular bed, extrinsic compression of the central pulmonary vessels and an intrinsic vasculopathy. Recognition of SAPH may be delayed as it can be masked by the clinical picture of underlying pulmonary sarcoidosis, and right heart catheter remains the gold-standard for diagnosis. Management of SAPH is based on reversal of resting hypoxaemia, treatment of comorbidities and treatment of the underlying sarcoidosis. The use of corticosteroids in SAPH is controversial. Specific PH therapy is not routinely recommended in SAPH as there are no successful placebo-controlled trials, although there is limited data to suggest that endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be useful.

摘要

肺动脉高压(PH)是结节病的一种公认的并发症。与 PH 相关的结节病(SAPH)患者的功能状态较差,对补充氧气的需求大于没有 PH 的结节病患者,并且更有可能被列入肺移植名单。PH 是等待肺移植的结节病患者死亡的独立危险因素。SAPH 的病理生理学很复杂,多种机制导致发病,包括肺血管床的纤维性破坏、中央肺血管的外在压迫和内在血管病变。由于 SAPH 可能被潜在的肺结节病的临床表现所掩盖,因此可能会延迟对 SAPH 的识别,而右心导管仍然是诊断的金标准。SAPH 的治疗基于纠正静息性低氧血症、治疗合并症和治疗潜在的结节病。在 SAPH 中使用皮质类固醇存在争议。由于没有成功的安慰剂对照试验,因此通常不建议在 SAPH 中使用特定的 PH 治疗,但有限的数据表明内皮素受体拮抗剂和磷酸二酯酶-5 抑制剂可能有用。

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