Abstract: The progress of diagnosis and treatment of autoimmune pancreatitis        WANG Li-wei*, LI Jian-ang，RONG Ye-fei, et al. *Department of General Surgery, the Fifth People’s Hospital, Suzhou215131，China
Corresponding author：LOU Wen-hui，E-mail：lou.wenhui@zs-hospital.sh.cn
Abstract    Autoimmune pancreatitis (AIP) is a rare disease, which was gradually cognized in recent years. AIP is a part of IgG4 Related Disease, histologically by numerous lymphoplasmacytic infiltrate and chronic fibrosis. The typical pancreatic imaging findings include "sausage like enlargement" or local mass formation in AIP patients, histopathology of the pancreas shows severe lymphoplasmacytic infiltration，especially IgG4-positive plasma cell infiltrations and storiform fibrosis. AIP patients indicate a good response to steroid therapy. AIP can be diagnosed and differentiated base on serum IgG4, image findings and histopathological features. Currently, the first-line treatment is corticosteroids, and Rituximab could be the treatment to high-risk patients with steroid intolerance. The Relapse of AIP is quite common. There is no consensus on the curative evaluation of IgG4 for steroid therapy, and IgG4 seems inoperative in curative evaluation. It is suggested to evaluate the therapeutic effect by clinical symptoms and imaging manifestations.

Key words: autoimmune pancreatitis, IgG4 related disease, lymphoplasmacytic sclerosing pancreatitis

摘要： 自身免疫性胰腺炎（AIP）是近年来逐渐被人们认知的一种少见病，属于血清免疫球蛋白G4（IgG4）相关性疾病的一种，病理学表现以胰腺炎症和纤维化为主。自身免疫性胰腺炎的病人在影像学上显示出胰腺“腊肠样肿大”或局部肿块形成，并伴随大量IgG4阳性浆细胞浸润和席纹状纤维化，此类病人对类固醇治疗反应良好。该疾病可通过检测血清IgG4，影像学改变和组织病理学特征进行诊断与鉴别。目前其一线治疗药物是皮质类固醇激素，对于激素不耐受的高风险病人可给予利妥昔单抗。自身免疫性胰腺炎复发率很高，IgG4对于激素治疗疗效评判尚未形成共识，IgG4在疗效评价上价值有限，建议参考临床症状及影像学表现评估治疗效果。

关键词: 自身免疫性胰腺炎, IgG4相关性疾病, 淋巴浆细胞性硬化性胰腺炎