Musculoskeletal and other extrapulmonary disorders in sarcoidosis

Musculoskeletal and other extrapulmonary disorders in sarcoidosis
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DOI:
10.1016/j.berh.2003.09.005
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发表时间:
2003-12-01
影响因子:
5.2
通讯作者:
Fayad, F
Fayad, F
中科院分区:
医学2区
文献类型:
--
作者:
Awada, H;Abi-Karam, G;Fayad, F

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结节病是一种多系统炎症性疾病,其起源仍未知,其特征是所有受影响器官均出现上皮样非干酪样肉芽肿。肉芽肿的形成是由 Th1 型反应引起的。导致疾病进展或自发消退的确切机制尚不清楚。家族聚集,以及结节病的表现和严重程度因种族背景而异,提示多基因起源仍有待确定。环境因素的贡献及其与遗传因素的相互作用仍有待证明。回顾临床表现、肌肉骨骼和其他肺外疾病以及患者的检查情况。结节病通常是一种良性疾病。皮质类固醇,无论是单独使用还是与其他药物联合使用,仍然是主要的治疗方法。明确定义谁需要治疗以及对特定患者使用什么治疗仍然存在争议。
Sarcoidosis is a multisystemic inflammatory disease, still of unknown origin, characterized by epithelioid non-caseating granuloma in all affected organs. Granuloma formation is lead by a ThI-type response. The exact mechanism that leads to either progression or spontaneous resolution of the disease is not known. Familial aggregation, and the variations in presentation and severity of sarcoidosis according to ethnic background, are suggestive of a polygenic origin that is still to be determined. The contribution of environmental factors, as well as their interactions with genetic factors, remains to be demonstrated. The clinical presentation, musculoskeletal and other extrapulmonary disorders, as well as patients work-up, are reviewed. Sarcoidosis is often a benign disease. Corticosteroids, either alone or in association with other drugs, are still the mainstay of treatment. Defining clearly who will need treatment and what treatment to be used in a particular patient remains controversial.