Manns Michael P, Vogel Arndt
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Germany.
Hepatology. 2006 Feb;43(2 Suppl 1):S132-44. doi: 10.1002/hep.21059.
In 1950, Waldenström was the first to describe a chronic form of hepatitis in young women. Subsequently, the disease was found to be associated with other autoimmune syndromes and was later termed "lupoid hepatitis" because of the presence of antinuclear antibodies. In 1965, it became designated by Mackay et al. as "autoimmune hepatitis" at an international meeting, at which the general concept of autoimmunity was endorsed by the scientific community. In the early 1960s and 1970s, the value of immunosuppressive therapy with glucocorticoids and/or azathioprine was well documented in several studies. The original association of autoimmune hepatitis (AIH) and HLA alleles, which has remarkably stood the test of time, was published in 1972. In the 1970s and 1980s, several autoantibodies were identified in patients with autoimmune hepatitis directed against proteins of the endoplasmatic reticulum expressed in liver and kidney and against soluble liver antigens. Subsequently, the molecular targets of these antibodies were identified and more precisely characterized. In the last two decades many additional pieces of the AIH puzzle have been collected leading to the identification of additional antibodies and genes associated with AIH and to the emergence of new therapeutic agents. Meanwhile, the immunoserological and genetic heterogeneity of AIH is well established and it has become obvious that clinical manifestations, disease behavior, and treatment outcome may vary by racial groups, geographical regions and genetic predisposition. Currently, the International Autoimmune hepatitis group is endorsing multi-center collaborative studies to more precisely define the features at disease presentation and to define prognostic indices and appropriate treatment algorithms. Given the importance of serological testing, the IAHG is also working on guidelines and procedures for more reliable and standardized testing of autoantibodies.
1950年，瓦尔登斯特伦首次描述了年轻女性中的一种慢性肝炎形式。随后，发现该疾病与其他自身免疫综合征有关，由于存在抗核抗体，后来被称为“狼疮样肝炎”。1965年，麦凯等人在一次国际会议上将其命名为“自身免疫性肝炎”，在这次会议上，自身免疫的总体概念得到了科学界的认可。在20世纪60年代初和70年代，几项研究充分证明了糖皮质激素和/或硫唑嘌呤免疫抑制疗法的价值。自身免疫性肝炎（AIH）与HLA等位基因的最初关联发表于1972年，该关联经受住了时间的考验。在20世纪70年代和80年代，在自身免疫性肝炎患者中发现了几种自身抗体，这些抗体针对肝脏和肾脏中表达的内质网蛋白以及可溶性肝抗原。随后，确定了这些抗体的分子靶点并进行了更精确的表征。在过去的二十年里，收集了许多有关AIH的其他信息，从而确定了与AIH相关的其他抗体和基因，并出现了新的治疗药物。与此同时，AIH的免疫血清学和遗传异质性已得到充分证实，很明显，临床表现、疾病行为和治疗结果可能因种族、地理区域和遗传易感性而异。目前，国际自身免疫性肝炎小组正在支持多中心合作研究，以更精确地定义疾病表现特征，并确定预后指标和适当的治疗方案。鉴于血清学检测的重要性，IAHG也在制定关于自身抗体更可靠和标准化检测的指南和程序。
