自身免疫性胰腺炎临床特征分析

An analysis of clinical characteristics of autoimmune pancreatitis

目的 探讨自身免疫性胰腺炎（autoimmune pancreatitis,AIP）的临床特点,提高对AIP的认识,降低误诊率.方法 分析2006-2009年期间在解放军总医院诊治的AIP患者的临床表现、影像及病理学特征和诊疗经过,并与1995-2005年期间的本院资料进行对比.结果 2006-2009年期间诊治8例AIP患者（男6例,女2例）,年龄35～69（52.4±9.4）岁.主要临床表现为黄疸6例（6/8）,腹痛5例（5/8）,伴有其他免疫疾病4例（4/8）.影像学见胰腺局部肿大3例（3/8）,弥漫性肿大5例（5/8）,其胰周均可见＂香蕉皮样＂囊状环,胰管不规则狭窄.病理检查见胰腺组织、胆管、涎腺及肝内可见大量淋巴细胞和浆细胞浸润及实质纤维化.7例（7/8）口服泼尼松治疗后临床表现、实验室及影像学表现均明显好转.我院1995-2005年期间资料显示,95.7%（22/23）误诊为胆道或胰腺癌,91.3%（21/23）行开腹手术,自2006年以后无一例出现误诊（0/8）.结论 AIP是全身免疫系统异常体现在胰腺的一种损伤,常伴发其他免疫性疾病,综合免疫指标检测、影像学、病理学检查及试验性糖皮质激素治疗等可获确诊.

Objective To increase the appreciation of manifestations in autoimmune pancreatitis （AIP） and to decrease misdiagnosis rate by investigating the clinical characteristics of AIP. Methods Clinical data were collected and laboratory ,imaging and histopathology were analyzed from the Chinese PLA General Hospital from 1995 to 2009. Patients with AIP were included in the study. Results Eight patients （male 6, female 2） aged 35-69 （52.4 ±9.4） years were diagnosed as AIP from 2006 to 2009. The main clinical manifestations include intermittent jaundice in 6 cases （6/8）, abdominal pain in 5 cases （5/8）,weight loss in 4 cases （4/8）, and accompanied with other diseases of immune system in 4 cases （4/8）.The imaging showed head of pancreas enlargement in 3 cases （3/8） and whole pancreas enlargement in 5 cases （5/8）. There is a ＂banana-peel like＂ ring around the pancreas and irregular stenosis of pancreatic duct. Massive lymphocytes and plasma cells infiltration and parenchymal fibrosis were shown in pancreatic tissues, bile duct,salivary gland and liver in pathology. Clinical manifestations, laboratory examinations and images in 7 cases （7/8） were improved after treatment with prednisone. Twenty-two of them were misdiagnosed as pancreatic or biliary carcinoma and 21 were performed laparotomy in the period of 1995-2005 （ 23 cases）. After 2006, however, the misdiagnosis rate significantly decreased from 95.7% （ 22/23 ） to 0. Conclusions AIP seems to be a systemic autoimmune disease rather than an isolated disorder,markedly overlapping with other autoimmune diseases. Definitive diagnosis can be improved by the detection of immune parameters and pathological examination.