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晚期肺结节病的治疗。

Management of Advanced Pulmonary Sarcoidosis.

机构信息

Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania.

Division of Pulmonary and Critical Care Medicine, Albany Medical College, Albany, New York; and.

出版信息

Am J Respir Crit Care Med. 2022 Mar 1;205(5):495-506. doi: 10.1164/rccm.202106-1366CI.

Abstract

The term "advanced sarcoidosis" is used for forms of sarcoidosis with a significant risk of loss of organ function or death. Advanced sarcoidosis often involves the lung and is described as "advanced pulmonary sarcoidosis" (APS), which includes advanced pulmonary fibrosis, associated complications such as bronchiectasis and infections, and pulmonary hypertension. Although APS affects a small proportion of patients with sarcoidosis, it is the leading cause of poor outcomes, including death. Here we review the major patterns of APS with a focus on the current management as well as potential approaches for improved outcomes for this most serious sarcoidosis phenotype.

摘要

“晚期结节病”用于描述有显著器官功能丧失或死亡风险的结节病形式。晚期结节病常累及肺部,并被描述为“晚期肺结节病”(APS),包括晚期肺纤维化、支气管扩张和感染等相关并发症以及肺动脉高压。尽管 APS 仅影响少数结节病患者,但它是导致不良预后(包括死亡)的主要原因。本文主要综述了 APS 的主要表现形式,重点讨论了目前的治疗方法以及改善这一最严重结节病表型的潜在方法。

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