IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy
IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy
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DOI：
10.1159/000090033
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发表时间：
2006-01-01
期刊：
影响因子：
3.6
通讯作者：
Okamoto, A.
中科院分区：
文献类型：
--
作者：
Kamisawa, T.;Nakajima, H.;Okamoto, A.
背景与目的：自身免疫性胰腺炎通常与血清IgG 4浓度升高有关，有时与硬化性胆管炎和干燥综合征有关。本研究旨在阐明IgG 4相关硬化性疾病的拟议实体。研究方法：受试者为自身免疫性胰腺炎（n = 26）、硬化性涎腺炎（n = 5）、慢性酒精性胰腺炎（n = 20）、涎石病（n = 34）、干燥综合征（n = 50）和原发性硬化性胆管炎（n = 3）患者。使用抗CD4-T、CD8-T和CD20-B细胞亚群的抗体和IgG 4对这些患者的各种器官和组织的切片进行免疫化学检查，并测量血清IgG 4浓度。结果如下：自身免疫性胰腺炎患者伴有硬化性胆管炎（n = 23）、硬化性涎腺炎（n = 2）、腹膜后纤维化（n = 2）、腹部（n = 5）和颈部（n = 4）淋巴结病。在胰腺、胰周腹膜后组织、肝外胆管、胆囊、胃、小唾液腺和腹腔淋巴结中，IgG 4阳性浆细胞的浸润较其他疾病多（p <0.01）。硬化性涎腺炎患者的小唾液腺和颌下腺也有这种浸润（P <0.01）。自身免疫性胰腺炎和硬化性涎腺炎患者血清IgG 4浓度显著升高（p <0.01）。结论：我们提出了一个新的临床病理实体的IgG 4相关的硬化性疾病合并硬化性胰腺炎，胆管炎，涎腺炎和腹膜后纤维化淋巴结病。版权所有（C）2006 S. Karger AG、巴塞尔和IAP。
Background and Aims: Autoimmune pancreatitis is usually associated with elevated serum IgG4 concentrations, and sometimes with sclerosing cholangitis and Sjogren's syndrome. This study aimed to elucidate the proposed entity of IgG4-related sclerosing disease. Methods: Subjects were patients with autoimmune pancreatitis (n = 26), sclerosing sialadenitis ( n = 5), chronic alcoholic pancreatitis ( n = 20), sialolithiasis ( n = 34), Sjogren's syndrome ( n = 50), and primary sclerosing cholangitis ( n = 3). Sections of various organs and tissues of these patients were examined immunohistochemically using antibodies to CD4-T, CD8-T, and CD20-B cell subsets and IgG4, and serum IgG4 concentrations were measured. Results: Patients with autoimmune pancreatitis were associated with sclerosing cholangitis ( n = 23), sclerosing sialadenitis ( n = 2), retroperitoneal fibrosis ( n = 2), and abdominal ( n = 5) and cervical ( n = 4) lymphadenopathy. They demonstrated infiltrations of more abundant IgG4-positive plasma cells in the pancreas, peripancreatic retroperitoneal tissues, extrahepatic bile duct, gallbladder, stomach, minor salivary gland, and abdominal lymph nodes compared with those of other diseases ( p < 0.01). Such infiltrations were also observed in the minor salivary gland and submandibular gland of patients with sclerosing sialadenitis ( p < 0.01). Serum IgG4 concentrations were significantly elevated in patients with autoimmune pancreatitis and sclerosing sialadenitis ( p < 0.01). Conclusion: We propose a new clinicopathological entity of IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Copyright (C) 2006 S. Karger AG, Basel and IAP.