Kamisawa Terumi, Sasaki Tsuneo
Depts. of Internal Medicine, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Japan.
Gan To Kagaku Ryoho. 2011 Mar;38(3):347-52.
IgG4-related sclerosing disease is a systemic disease histologically characterized by extensive T lymphocytes and IgG4-positive plasma cell infiltration of various organs. Major clinical manifestations are apparent in the pancreas (autoimmune pancreatitis), bile duct (sclerosing cholangitis), gallbladder (sclerosing cholecystitis), salivary gland (sclerosing sialadenitis), and retroperitoneum (retroperitoneal fibrosis), in which tissue fibrosis with obliterative phlebitis is pathologically induced. Autoimmune pancreatitis is a pancreatic lesion and its extrapancreatic lesions are organs reflecting an IgG4-related sclerosing disease. In some cases, only one or two organs are clinically involved, while in others three or four organs are affected. The disease occurs predominantly in elderly males, is frequently associated with lymphadenopathy, and responds well to steroid therapy. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery. Some cases of autoimmune pancreatitis were reportedly associated with pancreatic cancer. Although no relationship between the two diseases is known, we showed frequent and significant K-ras mutations in the pancreas, the bile duct, and the gallbladder in autoimmune pancreatitis.
IgG4相关硬化性疾病是一种全身性疾病，其组织学特征为各种器官广泛的T淋巴细胞和IgG4阳性浆细胞浸润。主要临床表现见于胰腺（自身免疫性胰腺炎）、胆管（硬化性胆管炎）、胆囊（硬化性胆囊炎）、唾液腺（硬化性涎腺炎）和腹膜后（腹膜后纤维化），其中病理上可诱导伴有闭塞性静脉炎的组织纤维化。自身免疫性胰腺炎是一种胰腺病变，其胰腺外病变是反映IgG4相关硬化性疾病的器官。在某些情况下，临床上仅累及一两个器官，而在其他情况下，则有三四个器官受累。该疾病主要发生于老年男性，常伴有淋巴结病，对类固醇治疗反应良好。由于初诊时经常怀疑为恶性肿瘤，因此在鉴别诊断中应考虑IgG4相关硬化性疾病，以避免不必要的手术。据报道，一些自身免疫性胰腺炎病例与胰腺癌有关。虽然两者之间的关系尚不清楚，但我们发现自身免疫性胰腺炎患者的胰腺、胆管和胆囊中K-ras突变频繁且显著。
