Recognition of other organ involvement might assist in the differential diagnosis of IgG4-associated
Recognition of other organ involvement might assist in the differential diagnosis of IgG4-associated
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识别其他器官受累可能有助于 IgG4 相关性的鉴别诊断
DOI：
10.1007/s00595-012-0278-6
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发表时间：
2012
期刊：
影响因子：
2.5
通讯作者：
Nowatari T
中科院分区：
文献类型：
--
作者：
Koike N;Onaya H;Nowatari T
igg4相关硬化性胆管炎（IAC）最近被定义为igg4相关的全身性疾病累及胆道。它常与自身免疫性胰腺炎相关，以胰腺肿大和胰管不规则狭窄为特征。然而，也有少数IAC病例没有明显的胰腺受累，其特征可能与胆管癌相似。我们报告两个罕见的igg4相关的硬化性胆管炎在肝门，模拟肝门胆管癌。当试图确定诊断时，我们应该考虑其他可能受累的器官，如胰腺、唾液腺、腹膜后、淋巴结和肾脏，以及慢性炎症改变。通过识别这些病变并测量血清IgG4，可以正确诊断IAC，从而避免对类固醇治疗有效的疾病进行不必要的大手术。
IgG4-associated sclerosing cholangitis (IAC) was recently defined as biliary involvement of IgG4-related systemic disease. It is frequently associated with autoimmune pancreatitis, characterized by pancreatic enlargement and irregular narrowing of the pancreatic duct. However, a few cases of IAC with no apparent pancreatic involvement have been described, the characteristics of which may mimic those of cholangiocarcinoma. We report two rare cases of IgG4-associated sclerosing cholangitis at the hepatic hilum, mimicking hilar cholangiocarcinoma. When trying to establish the diagnosis, we should consider other organs that could be involved, such as the pancreas, salivary glands, retroperitoneum, lymph nodes, and kidneys, as well as chronic inflammatory changes. By recognizing these lesions and measuring serum IgG4, IAC can be diagnosed correctly, thereby avoiding unnecessary major surgery for a condition that is treated effectively by steroid therapy.