Williams W J
Beitr Pathol. 1977 Aug;160(4):325-36. doi: 10.1016/s0005-8165(77)80117-0.
Sarcoidosis is defined as a multisystem disorder characterised by the finding of epithelioid cell granulomas in more than one system. Diagnosis is aided by the use of the Kveim Siltzbach skin test and the development of an "in vitro" Kmif test is discussed. Despite extensive researches the causative agent(s) remains unknown. The granulomas, morphologically, on light and electron microscopy and histochemistry may be indistinguishable from those caused by known agents. Inclusion bodies are also non specific. Central necrosis is rare, and can be usually distinguished from caseation. The close relationship between the monocyte derived, epithelioid cells and lymphocytes is emphasised. Evidence is accumulating that epithelioid cells in sarcoid type granulomas are primarily synthesising rather than phagocytic cells. The products are considered to be mucoglycoproteins and may have both local and systemic actions. Locally it is suggested that the products may be lymphokines which react with associated thymic derived (T) lymphocytes and mononuclear cells and thus play a role in perpetuating the granulomas. Epithelioid cells may also be a source of circulating T lymphocyte function depressants. It has further been suggested that epitheloid cells are the source of the raised angiotensin converting enzyme found in sarcoid sera. Study of epithelioid cell granulomas in sarcoidosis, despite the disappointing lack of evidence of a causative sarcoid agent(s), is thus of considerable interest in furthering knowledge of many diseases characterised by these curious cellular foci.
结节病被定义为一种多系统疾病，其特征是在多个系统中发现上皮样细胞肉芽肿。Kveim Siltzbach皮肤试验有助于诊断，文中还讨论了“体外”Kmif试验的开发情况。尽管进行了广泛研究，但致病因素仍然未知。从形态学上看，肉芽肿在光镜、电镜和组织化学下可能与已知病原体引起的肉芽肿无法区分。包涵体也不具有特异性。中央坏死很少见，通常可与干酪样坏死区分开来。文中强调了单核细胞衍生的上皮样细胞与淋巴细胞之间的密切关系。越来越多的证据表明，结节病型肉芽肿中的上皮样细胞主要是合成细胞而非吞噬细胞。其产物被认为是粘糖蛋白，可能具有局部和全身作用。在局部，有人认为这些产物可能是淋巴因子，它们与相关的胸腺衍生（T）淋巴细胞和单核细胞发生反应，从而在肉芽肿的持续存在中发挥作用。上皮样细胞也可能是循环T淋巴细胞功能抑制剂的来源。还有人进一步提出，上皮样细胞是结节病血清中血管紧张素转换酶升高的来源。因此，尽管令人失望地缺乏结节病病原体的证据，但对结节病中上皮样细胞肉芽肿的研究对于增进对许多以这些奇特细胞灶为特征的疾病的认识具有相当大的意义。
