Identification and characterization of IgG4-associated autoimmune hepatitis
Identification and characterization of IgG4-associated autoimmune hepatitis
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DOI：
10.1111/j.1478-3231.2009.02092.x
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发表时间：
2010-02-01
影响因子：
6.7
通讯作者：
Chiba, Tsutomu
中科院分区：
文献类型：
--
作者：
Chung, Hobyung;Watanabe, Tomohiro;Chiba, Tsutomu
背景自身免疫性肝炎（AIH）和自身免疫性胰腺炎（AIP）具有共同的临床和病理特征，例如高血清免疫球蛋白（IG）G和自身抗体水平以及淋巴浆细胞浸润，表明存在常见的免疫学异常。然而，很少有人知道的可能参与的IgG 4，标志性的AIP，在AIH.AimsIn这项研究中，我们研究了是否IgG 4反应有助于组织病理学和临床发现在AIH.MethodsLiver切片从26例AIH患者，10例原发性胆汁性肝硬化（PBC），对3名原发性硬化性胆管炎（PSC）患者和20名丙型肝炎病毒（HCV）感染的慢性肝炎患者进行IgG 4免疫染色。结果26例AIH患者肝组织中9例IgG4染色阳性，而PBC患者肝组织中10例IgG4染色阳性，PSC患者肝组织中3例IgG4染色阳性，HCV肝炎患者肝组织中20例IgG4染色阳性。IgG4阳性AIH患者血清IgG水平也升高。与IgG 4阴性AIH患者相比，IgG 4阳性AIH患者肝脏中T细胞、B细胞和浆细胞数量明显增加。IgG4阳性AIH患者也表现出显着的反应泼尼松龙therapy.ConclusionsAIH可分为IgG4相关型或IgG4非相关型，前者表现出显着的反应泼尼松龙治疗。
BackgroundAutoimmune hepatitis (AIH) and autoimmune pancreatitis (AIP) share clinical and pathological features such as high serum levels of immunoglobulin (Ig) G and autoantibodies, and lymphoplasmacytic infiltration, suggesting the presence of common immunological abnormalities. However, little is known about the possible involvement of IgG4, a hallmark of AIP, in AIH.AimsIn this study, we examined whether the IgG4 response contributes to the histopathological and clinical findings in AIH.MethodsLiver sections from 26 patients with AIH, 10 patients with primary biliary cirrhosis (PBC), three patients with primary sclerosing cholangitis (PSC) and 20 chronic hepatitis patients with hepatitis C virus (HCV) infection were immunostained for IgG4. We investigated the relationship among the histopathology, the responses to steroid therapy and the IgG4 staining.ResultsNine of the 26 liver specimens from patients with AIH showed positive staining for IgG4 whereas none of the 10 samples from patients with PBC, the three samples from patients with PSC or the 20 samples from patients with HCV hepatitis were positive. Patients with IgG4-positive AIH also showed increased serum levels of IgG. The numbers of T cells, B cells and plasma cells were significantly increased in the livers of patients with IgG4-positive AIH as compared with those patients with IgG4-negative AIH. Patients with IgG4-positive AIH also showed a marked response to prednisolone therapy.ConclusionsAIH may be classified into either an IgG4-associated type or an IgG4 non-associated type with the former showing a marked response to prednisolone treatment.