探讨IgG4相关自身免疫性胰腺炎（AIP）的临床特点。

回顾性研究2003年1月至2012年12月在中山大学孙逸仙纪念医院肝胆胰外科接受诊治并经病理学确诊的12例IgG4相关AIP患者临床资料。所有患者均签署知情同意书，符合医学伦理学规定。患者均为男性，平均年龄为（54±13）岁，均被误诊为胰头癌行胰头十二指肠切除术。收集患者临床表现及实验室、影像学、病理学检查等资料。患者术后接受随访，观察治疗情况及疗效。

12例患者中腹痛7例、黄疸7例、消瘦6例、无明显症状1例，合并糖尿病3例、慢性下颌下腺炎1例。患者血、尿淀粉酶均正常，血GGT升高12例、糖链抗原19-9（CA19-9）轻度升高9例、癌抗原（CA）125升高3例、癌胚抗原（CEA）升高2例。增强CT示胰头局部肿大11例，伴胰管轻度扩张3例。MRI示胰体呈"腊肠样"改变4例，胰周呈"包鞘样"改变5例。磁共振胰胆管成像（MRCP）示胰头段胰管及胆总管下段狭窄，其中4例远端胰管轻度扩张。影像学检查未发现局部侵犯。病理学检查表现为淋巴浆细胞硬化性胰腺炎，免疫组织化学方法（免疫组化法）染色IgG4阳性。12例术后患者间断出现腹痛，其中7例需服用止痛药。1例患者经泼尼松治疗后症状缓解。

IgG4相关AIP临床表现与胰腺癌相似，易误诊为胰腺癌。其主要临床特点为血CA19-9轻度升高；影像学检查见胰头局部肿大、胰体呈"腊肠样"、胰周呈"包鞘样"改变，且无局部侵犯；病理学检查为淋巴浆细胞硬化性胰腺炎，免疫组化法染色IgG4阳性；肾上腺皮质激素治疗有效。

To investigate the clinical features of IgG4 related autoimmune pancreatitis (AIP).

Clinical data of 12 patients pathologically diagnosed as IgG4 related AIP in Department of Hepatopancreatobiliary Surgery, Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2003 to December 2012 were retrospectively analyzed. The informed consents of all patients were obtained and the ethical committee approval was received. All the patients were males with a mean age of (54±13) years old. All were misdiagnosed as pancreatic head carcinoma and received pancreatoduodenectomy. Data of the clinical manifestations, laboratory, imaging and pathological examinations were collected. The patients were followed up after operations, the treatment and outcome were studied.

The clinical manifestations of the 12 patients were abdominal pain (n=7), jaundice(n=7), emaciation(n=6) and no obvious symptom (n=1). Three cases were combined with diabetes, 1 case with chronic sialadenitis of submandibular gland. The patient's blood and urine amylase were normal. Blood gamma-glutamyl transpeptidase (GGT) increase was observed in 12 cases. Mild increase of carbohydrate antigen 19-9 (CA19-9) was observed in 9 cases. Cancer antigen (CA) 125 increase was observed in 3 cases, and carcinoembryonic antigen (CEA) increase in 2 cases. Through contrast-enhanced CT, partial pancreatic head enlargement was observed in 11 cases, in which 3 cases were observed combining with mild expansion of pancreatic duct. Through magnetic resonance imaging (MRI), sausage-like change of pancreas body was observed in 4 cases, peripancreatic sheath-like change in 5 cases. Through magnetic resonance cholangiopancreatography (MRCP), stenoses in the pancreatic duct of pancreatic head segment and distal common bile duct were observed including 4 cases of mild expansion in the distal pancreatic duct. Local invasion was not observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis was determined by pathological examination. Positive expression of IgG4 was observed by immunohistochemistry. Twelve cases suffered from discontinuous abdominal pain after operations, in which 7 cases needed acesodyne. One case relieved after using prednisone.

Clinical manifestations of IgG4 related AIP are similar to pancreatic carcinoma and can be misdiagnosed as pancreatic carcinoma easily. The main clinical features are mild increase of blood CA19-9. Partial pancreatic head enlargement, pancreas body sausage-like change, peripancreatic sheath-like change, and no local invasion are observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis is determined by pathological examination. Positive expression of IgG4 is observed by immunohistochemistry. Adrenocortical hormone treatment is effective.