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当前对结节病的治疗 I:肺部、心脏和神经系统表现。

Current management of sarcoidosis I: pulmonary, cardiac, and neurologic manifestations.

机构信息

Department of Medicine, University of Colorado School of Medicine, Division of Rheumatology, Aurora, Colorado, USA.

出版信息

Curr Opin Rheumatol. 2018 May;30(3):243-248. doi: 10.1097/BOR.0000000000000489.

Abstract

PURPOSE OF REVIEW

Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation of multiple organ systems. Pulmonary, cardiac, and neurologic involvements have the worst prognosis. Current recommendations for the therapeutic management and follow-up of sarcoidosis involving these critical organs will be reviewed.

RECENT FINDINGS

In those sarcoidosis patients requiring immunosuppressive therapy, corticosteroids are used first at varying doses depending on the presenting manifestation. Patients with symptomatic pulmonary, cardiac, or neurologic involvement will be maintained on corticosteroids for at least a year. Many require a second immunosuppressive agent with methotrexate used most commonly. Anti-tumor necrosis factor agents, especially infliximab, are effective and recommendations for their use have been proposed.

SUMMARY

Evidence-based treatment guidelines do not exist for most sarcoidosis clinical manifestations. Therefore, clinical care of these patients must rely on expert opinion. Patients are best served by a multidisciplinary approach to their care. Future research to identify environmental triggers, genetic associations, biomarkers for treatment response, and where to position new steroid-sparing immunosuppressive agents is warranted.

摘要

目的综述

结节病是一种全身性疾病,其特征是多器官系统的非干酪样肉芽肿性炎症。肺部、心脏和神经系统受累的预后最差。现将对涉及这些重要器官的结节病的治疗管理和随访的最新建议进行回顾。

最近的发现

在那些需要免疫抑制治疗的结节病患者中,根据临床表现的不同,皮质类固醇的使用剂量也不同。有症状的肺部、心脏或神经系统受累的患者至少需要服用皮质类固醇一年。许多人需要使用第二种免疫抑制剂,最常用的是甲氨蝶呤。抗肿瘤坏死因子(TNF)药物,尤其是英夫利昔单抗,是有效的,并且已经提出了使用这些药物的建议。

总结

大多数结节病的临床表现都没有基于证据的治疗指南。因此,对这些患者的临床护理必须依赖专家意见。多学科的方法对这些患者的护理最有效。有必要开展未来的研究,以确定环境触发因素、遗传关联、治疗反应的生物标志物以及新的类固醇保肺免疫抑制剂的定位。

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