Rohart C, Badelon I, Fajnkuchen F, Nghiem-Buffet S, Chaine G
Service d'ophtalmologie, Hôpital Avicenne, Université Paris XIII, Bobigny, France.
J Fr Ophtalmol. 2008 Sep;31(7):683-91. doi: 10.1016/s0181-5512(08)74382-1.
Granulomatosis lesions occurring after diagnosis of primary or secondary immunodeficiency are not accidental and have been described in a small number of patients suffering from various diseases: common variable immunodeficiency (CVID), malignancy (lymphoma and solid tumors), and acquired immunodeficiency syndrome (AIDS). Two types of granulomatosis can appear: true sarcoidosis and sarcoid-like reaction. We report four patients, two with CVID and two with malignancy, in whom clinical granulomatosis appeared a few months to a few years after diagnosis of immunodeficiency. They developed noncaseating granulomas of the lung, spleen and liver associated with conjunctival granulomas and bilateral panuveitis. The granulomatous disorder was diagnosed after immunodeficiency on histopathological studies revealing noncaseating granulomas. Causation agents such as infectious organisms and environmental compounds were excluded. The relationship between sarcoid-like reaction, true sarcoidosis and immunodeficiency is discussed. The underlying pathophysiology responsible for the association between granuloma formation and immunodeficiency in the same patient remains obscure. It may be quite difficult to distinguish true sarcoidosis and sarcoid-like reaction. It is possible that these two entities are the clinical extremes of a common pathological process.
在原发性或继发性免疫缺陷诊断后出现的肉芽肿性病变并非偶然，在少数患有各种疾病的患者中已有描述：常见可变免疫缺陷（CVID）、恶性肿瘤（淋巴瘤和实体瘤）以及获得性免疫缺陷综合征（AIDS）。可出现两种类型的肉芽肿：真性结节病和结节病样反应。我们报告了4例患者，其中2例患有CVID，2例患有恶性肿瘤，他们在免疫缺陷诊断后的数月至数年出现了临床肉芽肿。他们出现了肺、脾和肝的非干酪样肉芽肿，伴有结膜肉芽肿和双侧全葡萄膜炎。在免疫缺陷后，经组织病理学研究发现非干酪样肉芽肿而诊断为肉芽肿性疾病。排除了感染性生物体和环境化合物等致病因素。讨论了结节病样反应、真性结节病与免疫缺陷之间的关系。同一患者中肉芽肿形成与免疫缺陷之间关联的潜在病理生理学仍不清楚。区分真性结节病和结节病样反应可能相当困难。这两种实体有可能是同一病理过程的临床极端表现。
