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结节病

Sarcoidosis.

作者信息

Sharma O P

机构信息

University of Southern California, School of Medicine, Los Angeles.

出版信息

Dis Mon. 1990 Sep;36(9):469-535. doi: 10.1016/0011-5029(90)90004-b.

Abstract

More than a century ago, Jonathan Hutchingson, a surgeon-dermatologist, identified the first case of sarcoidosis at King's College, London. The disease is now known as a commonplace multisystem disorder characterized by the formation of noncaseating granulomata. The diagnosis of sarcoidosis is established by recognizing clinicoradiologic findings and providing histologic evidence of non-caseating granuloma. Serum angiotensin converting enzyme levels are high in about two thirds of the patients and hypercalcemia is a feature in one of every ten victims of sarcoidosis. Immunologic abnormalities include depression of cutaneous delayed-type hypersensitivity, accumulation of T-cells at the site of activity, hyperactive B-cells, and the presence of circulating immune complexes. The course and prognosis of the disease usually correlate with the mode of onset. An acute onset with erythema nodosum indicates a good prognosis and spontaneous resolution; whereas, an insidious onset may be followed by relentless, progressive fibrosis. Mortality and morbidity are caused by pulmonary fibrosis, cardiac arrhythmias, renal failure, neurologic involvement, and blindness. Corticosteroids and chloroquine relieve symptoms and suppress inflammation and granuloma formation.

摘要

一个多世纪前,外科皮肤科医生乔纳森·哈钦森在伦敦国王学院确诊了首例结节病病例。如今,这种疾病被认为是一种常见的多系统疾病,其特征是形成非干酪样肉芽肿。结节病的诊断是通过识别临床放射学表现并提供非干酪样肉芽肿的组织学证据来确立的。大约三分之二的患者血清血管紧张素转换酶水平升高,每十名结节病患者中就有一人出现高钙血症。免疫异常包括皮肤迟发型超敏反应降低、活动部位T细胞积聚、B细胞活性增强以及循环免疫复合物的存在。该病的病程和预后通常与发病方式相关。伴有结节性红斑的急性发病提示预后良好且可自发缓解;而隐匿性发病可能随后出现持续的进行性纤维化。死亡率和发病率是由肺纤维化、心律失常、肾衰竭、神经受累和失明引起的。皮质类固醇和氯喹可缓解症状并抑制炎症和肉芽肿形成。

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