Gras M, van Kemenade F J, van Maarsseveen A C M Th, Alberts C
VU Medisch Centrum, afd. Pathologie, Postbus 7057, 1007 MB Amsterdam.
Ned Tijdschr Geneeskd. 2003 Jan 25;147(4):150-5.
Sarcoidosis is a multi-systemic disease of unknown aetiology, immunopathologically and histologically characterised by a macrophage/T-helper I cell-mediated non-caseating granulomatous inflammation process. In the development of granulomas, an imbalance between pro-inflammatory and anti-inflammatory cytokines plays an important role, possibly initiated by an as yet unidentified (exogenous) stimulus. The clinical outcome of the disease process is unpredictable, and appears to be determined by the cytokine production of the inflammatory cells in the granuloma. Fifty to seventy percent of the patients recover without medication within a period of time which cannot be predicted, the other 30 to 50% of patients are treated during the course of the disease with corticosteroids (with varying degrees of success), either alone or in combination with cytostatic or immunosuppressive agents. Based on recent developments in the field of immunomodulation and on current knowledge of the immunopathogenesis of sarcoidosis, there appear to be opportunities for specific immunotherapy which should be evaluated in controlled studies.
结节病是一种病因不明的多系统疾病,在免疫病理学和组织学上的特征是巨噬细胞/T辅助1细胞介导的非干酪性肉芽肿性炎症过程。在肉芽肿的形成过程中,促炎细胞因子和抗炎细胞因子之间的失衡起着重要作用,这可能由一种尚未确定的(外源性)刺激引发。疾病过程的临床结果不可预测,似乎由肉芽肿中炎症细胞的细胞因子产生所决定。50%至70%的患者在无法预测的一段时间内无需药物治疗即可康复,另外30%至50%的患者在疾病过程中接受皮质类固醇治疗(成功率各不相同),单独使用或与细胞毒性或免疫抑制剂联合使用。基于免疫调节领域的最新进展以及对结节病免疫发病机制的现有认识,似乎有机会进行特异性免疫治疗,应在对照研究中进行评估。