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终末期结节病的管理:肺动脉高压和肺移植。

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation.

机构信息

Advanced Lung Disease and Transplant Program, Dept of Medicine, Inova Fairfax Hospital, Falls Church, VA 22042, USA.

出版信息

Eur Respir J. 2012 Jun;39(6):1520-33. doi: 10.1183/09031936.00175511. Epub 2012 Jan 12.

Abstract

Sarcoidosis is not only a multisystem, but also a multinational disease that is prevalent throughout the world, including Europe, the USA and Japan. Lung involvement in sarcoidosis is seemingly invariable, with up to 95% of patients manifesting some form of pulmonary disease during the course of their lifetime. The natural history of sarcoidosis in the lung is quite variable and spans the spectrum from spontaneous resolution to advanced fibrocystic disease in ~5% of cases. Advanced sarcoidosis will be the subject of this review with a special focus on pulmonary hypertension and lung transplantation as a last-resort treatment option for some patients with end-stage disease.

摘要

结节病不仅是一种多系统疾病,也是一种全球性疾病,在包括欧洲、美国和日本在内的世界各地都很流行。结节病的肺部受累似乎是不可避免的,高达 95%的患者在其一生中都会出现某种形式的肺部疾病。结节病在肺部的自然病程变化很大,从自发缓解到约 5%的病例中出现晚期纤维囊性疾病不等。晚期结节病将是本次综述的主题,特别关注肺动脉高压和肺移植,作为一些终末期疾病患者的最后治疗选择。

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