Humbert P, Dupond J L
Service de Médecine Interne, Centre Hospitalier Universitaire Jean Minjoz, Besançon.
Ann Med Interne (Paris). 1988;139(3):159-68.
The possibility of three or more autoimmune diseases occurring in the same patient cannot be fortuitous and suggests a pathogenic relationship between each of them. In the light of 4 personal cases, the authors have recorded 87 reports of such associations in the literature, an analysis of which leads them to propose a classification of three types of multiple autoimmune syndrome. The grouping of these syndromes under a single heading should make the research and analysis of these morbid associations easier. Moreover, the classification adopted by the authors allows a more precise definition of patients with at least two autoimmune diseases and so helps to recognize the onset of a third autoimmune disease at a later date. Multiple autoimmune syndromes can be classified in 3 groups according to the prevalence of their associations one with another. Type I comprises myasthenia, thymoma, polymyositis and giant cell myocarditis, this association having a single pathogenic mechanism. Type II includes the Sjögren's syndrome, rhumatoid arthritis, primary biliary cirrhosis, scleroderma and autoimmune thyroid disorders. Type III groups together 10 autoimmune diseases (autoimmune thyroid disease, myasthenia and/or thymoma, Sjögren's syndrome, pernicious anaemia, idiopathic thrombocytopaenic purpura, Addison's disease, insulin-dependent diabetes, vitiligo, autoimmune haemolytic anaemia, systemic lupus erythematosus) for which a genetic predisposition (phenotype HLA B8 and/or DR3 or DR5) seems to be an important factor.
同一患者出现三种或更多自身免疫性疾病并非偶然,这表明它们之间存在致病关系。根据4例个人病例,作者在文献中记录了87例此类关联的报告,对其进行分析后,他们提出了三种类型的多重自身免疫综合征的分类。将这些综合征归为一个类别应有助于对这些病态关联进行研究和分析。此外,作者采用的分类方法能够更精确地定义患有至少两种自身免疫性疾病的患者,从而有助于在日后识别第三种自身免疫性疾病的发病情况。多重自身免疫综合征可根据它们相互关联的发生率分为3组。I型包括重症肌无力、胸腺瘤、多发性肌炎和巨细胞心肌炎,这种关联具有单一的致病机制。II型包括干燥综合征、类风湿关节炎、原发性胆汁性肝硬化、硬皮病和自身免疫性甲状腺疾病。III型将10种自身免疫性疾病(自身免疫性甲状腺疾病、重症肌无力和/或胸腺瘤、干燥综合征、恶性贫血、特发性血小板减少性紫癜、艾迪生病、胰岛素依赖型糖尿病、白癜风、自身免疫性溶血性贫血、系统性红斑狼疮)归为一组,对于这些疾病,遗传易感性(HLA B8和/或DR3或DR5表型)似乎是一个重要因素。