Wu Li-li, Li Wen
Department of Gastroenterology and Hepatology, Chinese PLA General Hospital, Beijing 100853, China.
Zhonghua Nei Ke Za Zhi. 2010 Nov;49(11):943-6.
To increase the appreciation of manifestations in autoimmune pancreatitis (AIP) and to decrease misdiagnosis rate by investigating the clinical characteristics of AIP.
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.
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.
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.
通过研究自身免疫性胰腺炎（AIP）的临床特征，提高对其临床表现的认识并降低误诊率。
收集中国人民解放军总医院1995年至2009年的临床资料，对实验室检查、影像学及组织病理学进行分析。纳入AIP患者进行研究。
2006年至2009年共诊断出8例AIP患者（男性6例，女性2例），年龄35 - 69岁（52.4±9.4岁）。主要临床表现为间歇性黄疸6例（6/8）、腹痛5例（5/8）、体重减轻4例（4/8），4例（4/8）伴有其他免疫系统疾病。影像学表现为胰腺头部肿大3例（3/8），全胰腺肿大5例（5/8）。胰腺周围有“香蕉皮样”环状影及胰管不规则狭窄。病理显示胰腺组织、胆管、唾液腺及肝脏有大量淋巴细胞和浆细胞浸润及实质纤维化。7例（7/8）患者经泼尼松治疗后临床表现、实验室检查及影像学表现均有改善。1995年至2005年期间，22例被误诊为胰腺癌或胆管癌，21例行剖腹手术（共23例）。然而，2006年后误诊率从95.7%（22/23）显著降至0。
AIP似乎是一种全身性自身免疫性疾病而非孤立性疾病，与其他自身免疫性疾病有明显重叠。通过检测免疫指标和病理检查可提高确诊率。
