自身免疫性胰腺炎误诊误治17例临床分析

Mis-diagnosis and mis-treatment of autoimmune pancreatitis： a clinical study of 17 cases

目的 总结自身免疫性胰腺炎（AIP）误诊误治的原因.方法 回顾性分析2005年5月至2010年7月间我院收治的17例有误诊误治经历的AIP患者的临床资料.结果 AIP的主要临床症状包括腹部轻度疼痛13例、进行性黄疸12例、发热6例、体重减轻9例.15例伴随一种或一种以上胰腺外脏器的自身免疫性疾病,包括过敏性鼻炎、颌下淋巴结肿大、颌下腺肿大、过敏性哮喘、类风湿性关节炎、干燥综合征、糖尿病、原发性硬化性胆管炎和自身免疫性肝炎.实验室检查方面,血清球蛋白增高11例、IgG增高14例、γ球蛋白增高13例、抗核抗体阳性13例、抗胰岛素IgG抗体阳性2例.影像学检查方面,胰腺弥漫性肿大15例,伴主胰管弥漫性狭窄或节段性狭窄、胰腺段胆总管狭窄、近端胆管扩张、胆囊增大;胰头、钩突局灶性肿大2例.17例中有13例误诊为胰腺癌,其中4例行胰十二指肠切除术,7例行胆肠吻合术,2例被认为肿瘤晚期失去治疗机会;4例被误诊为普通慢性胰腺炎.确诊AIP后,17例患者均接受糖皮质激素治疗,效果显著.随访6～45个月（平均15个月）,4例复发,再次子糖皮质激素治疗效果好.随访期间未发现胰腺癌.结论 AIP 临床特点与胰腺癌相似,加之我国现阶段对AIP认识不足、重视不够,是导致误诊和误治的主要原因.

Objective To summarize the reasons of mis-diagnosis and mis-treatment of autoimmune pancreatitis （AIP）. Methods Clinical data of 17 patients with AIP,who were admitted to the hospital from May 2005 to July 2010 and experienced mis-diagnosis and mis-treatment, were retrospectively analyzed. Results The main clinical manifestations included epigastric pain （13 cases）,progressive obstructive jaundice （12 cases）, fever （6 cases） and weight loss （9 cases）. Fifteen patients had extrapancreatic organ involvemnet, including allergic rhinitis, swelling of lymphoglandulae submaxillares, swelling of submaxillary gland, allergic asthma, rheumatoid arthritis, Sjogren syndrome, diabetes mellitus, primary sclerosing cholangitis and autoimmune hepatitis. Of these 17 cases, 11 cases presented with high serum globulin, 14 cases with high serum IgG, 13 cases with high serum γ-globulin, 13 cases with positive anti-nuclear antibody and 2 cases with positive anti-insulin IgG antibody. The abdominal imaging demonstrated that 15 patients had diffuse enlargement of the pancreas with diffuse or segmental narrowing of main pancreatic duct, narrowing of the intrapancreatic common bile duct, dilation of the proximal biliary duct and gallbladder enlargement. Focal enlargement of the pancreas was found in 2 cases. Thirteen cases were misdiagnosed as pancreatic carcinoma. Among them, 4 cases underwent pancreaticoduodenectomy and 7 cases underwent choledochojejunostomy. Two cases were misdiagnosed as end stage of cancer that lost therapeutic chance. Another 4 cases were misdiagnosed as chronic pancreatitis. Steroid therapy was administered in all patients with satisfactory response. All patients were followed-up for 15 months （ranged from 6 months to 45 months）, and recurrence was found in 4 cases. Satisfactory response was found in patients treated with steroid for the second time. No pancreatic cancer was found in these patients in the follow up period. Conclusion The main causes of mis-diagnosis and mis-treatment of AIP may be contributed by difficulty in differentiating AIP from pancreatic carcinoma based on clinical manifestations and inadequate knowledge of AIP as well as insufficient attention to AIP in China.