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结节病管理的当前及新出现的策略

Current and emerging strategies for the management of sarcoidosis.

作者信息

White Eric S, Lynch Joseph P

机构信息

University of Michigan Medical Center, Division of Pulmonary and Critical Medicine, Department of Internal Medicine, 6301 MSRB III/0642, 1150 W. Medical Center Drive, Ann Arbor, MI 48109-0642, USA.

出版信息

Expert Opin Pharmacother. 2007 Jun;8(9):1293-311. doi: 10.1517/14656566.8.9.1293.

Abstract

Sarcoidosis is a systemic inflammatory disorder of unknown etiology. Although any organ may be involved, the lungs are most frequently affected. The clinical course of the disease is highly variable, with up to two-thirds of untreated patients experiencing spontaneous remission within 12-24 months of onset of symptoms. When therapy is required, corticosteroids are considered standard, but studies demonstrating their ability to modify the long-term outcome in this disease are lacking. Often, the myriad of adverse side effects of corticosteroids necessitate the addition of immunosuppressants, cytotoxic agents or biologic therapies to maintain disease remission. Unfortunately, optimal therapeutic regimens have not been described. Patients who do not respond to therapy often experience progressive fibrotic changes and end-organ damage, which ultimately may result in significant morbidity or death. Agents commonly used to treat patients with sarcoidosis and emerging therapeutic options are discussed.

摘要

结节病是一种病因不明的全身性炎症性疾病。尽管任何器官都可能受累,但肺部最常受到影响。该病的临床病程差异很大,多达三分之二未经治疗的患者在症状出现后的12 - 24个月内会自发缓解。当需要治疗时,皮质类固醇被视为标准治疗药物,但缺乏证明其能改变该病长期预后的研究。通常,皮质类固醇的众多不良副作用使得必须添加免疫抑制剂、细胞毒性药物或生物疗法以维持疾病缓解。不幸的是,尚未描述出最佳治疗方案。对治疗无反应的患者常出现进行性纤维化改变和终末器官损害,最终可能导致严重的发病或死亡。本文将讨论常用于治疗结节病患者的药物及新出现的治疗选择。

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