IgG4相关性自身免疫性胰腺炎

Immunoglobulin G4 related autoimmune pancreatitis

自身免疫性胰腺炎(AIP)是一种少见的胰腺慢性炎症性疾病,属于IgG4相关性疾病谱。组织学表现为胰管周围大量淋巴细胞浆细胞浸润、致密纤维化、闭塞性静脉炎、IgG4阳性浆细胞明显增多。临床表现为复发性胰腺炎、梗阻性黄疸,伴胆管、涎腺等其他器官受累。实验室可见血清IgG4明显升高。影像学表现为胰腺腊肠样外观,伴主胰管弥漫不规则狭窄。AIP对激素治疗敏感,但容易复发,对疾病复发的患者可再次激素治疗或联合免疫调节药物治疗。

Autoimmune pancreatitis （AIP） is a rare form of chronic pancreatitis which belongs to the spectrum of IgG4 related diseases. AIP is characterized by lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and increasing of IgG4 ＋ plasma cells. Patients with AIP presented clinically with recurrent pancreatitis and obstructive jaundice,usually with symptoms involving bile duct and salivary glands and so on. Serum IgG4 level is often elevated. The main imaging appearance of AIP is a ＂sausage-shaped pancreas＂ and multiple stricture of the main pancreatic duct. AIP responds dramatically to glucocorticosteroid but relapses easily. In relapsed cases, re-administration of steroid or in combination with immunosuppressant is effective.