Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?
Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?
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DOI：
10.1016/s0016-5107(05)00561-4
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发表时间：
2005-07-01
影响因子：
7.7
通讯作者：
Miyagawa, S
中科院分区：
文献类型：
--
作者：
Hamano, H;Kawa, S;Miyagawa, S
背景资料：自身免疫性胰腺炎被命名为硬化性胰胆管炎，因为这种疾病显示出高患病率的胆管病变。我们在此提出的临床特点不寻常的情况下，显示占主导地位的胆管病变和模仿浸润性肝门cholangiocarcinoma.Methods：临床和病理结果的3例免疫球蛋白（IG）G4相关的硬化性胆管炎谁没有明显的胰腺病变可比的自身免疫性胰腺炎进行了分析。观察：所有患者均为中年或老年人，血清IgG 4浓度轻微升高，并显示胆管系统长段狭窄，类似浸润性肝门胆管癌，但无显著胰腺变化。第一例患者接受皮质类固醇治疗，改善了胆管狭窄。第二例患者根据胆管癌的诊断接受了手术。在第三例患者中，胆管狭窄在引流术后1个月自发逆转。所有患者的胆管的病理结果显示显着的淋巴浆细胞浸润，包括丰富的IgG 4轴承浆cells.Conclusions：使用IgG 4免疫染色活检标本的胆管可以确定存在的皮质类固醇反应性淋巴浆细胞硬化性胆管炎。
Background: Autoimmune pancreatitis has been designated as sclerosing pancreatocholangitis, because this disease shows a high prevalence of bile-duct lesions. We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas.Methods: Clinical and pathologic findings of 3 patients with immunoglobulin (Ig) G4 related sclerosing cholangitis who had no apparent pancreatic lesions comparable with autoimmune pancreatitis were analyzed.Observations: All patients were middle-aged or elderly individuals with slightly elevated serum IgG4 concentrations and showed long-segment narrowing of the bile-duct system, mimicking infiltrating hilar cholangiocarcinoma without significant pancreatic change. The first patient was treated with a corticosteroid, resulting in amelioration of the narrowing of the bile duct. The second patient underwent surgery based on a diagnosis of cholangiocarcinoma. In the third patient, the bile-duct stricture reversed spontaneously 1 month after the drainage procedure. Pathologic findings of the bile ducts for all patients disclosed significant lymphoplasmacytic infiltration, including abundant IgG4-bearing plasma cells.Conclusions: The use of IgG4 immunostaining in biopsy specimens of the bile duct may identify the presence of corticosteroid-responsive lymphoplasmacytic sclerosing cholangitis.