自身免疫性胰腺炎21例临床病理分析

Autoimmune pancreatitis of 21 cases: histopathological features and clinical relevance

目的探讨局限性自身免疫性胰腺炎(autoimmune pancreatitis,AIP)的临床病理学特点、诊断及鉴别诊断。方法收集21例肿块性胰腺炎,病理学形态符合AIP,查找临床资料,分析其与临床病理特征的关系,并复习相关文献。结果21例患者中,男性13例,女性8例;患者年龄37~74岁,平均58.8岁。10例患者有皮肤、巩膜黄染,5例查体发现胰腺占位,6例以腹痛、腹部不适就诊。18例术前行血清IgG4检测。眼观:21例均发现胰腺肿块,直径3~7 cm,切面灰白、灰黄色,实性,质韧,与周围边界不清。镜下见导管周有较多淋巴细胞及浆细胞围绕,伴纤维组织席纹样增生,肿块内呈不同程度慢性胰腺炎表现;胰腺肿块内及胰腺周可见静脉炎;6例导管管腔内见较多中性粒细胞浸润并伴导管上皮破坏。免疫表型:10例IgG4阳性(阳性浆细胞数超过10/HPF);实验室检查IgG4超出正常上限2倍有6例患者,2例术前未检查IgG4,2例IgG4增高但不足2倍。结论肿块性AIP仅依据临床和影像学表现不易与胰腺癌鉴别,病理形态表现为淋巴浆细胞性硬化性胰腺炎,伴或不伴中性粒细胞管腔内聚集。手术标本经免疫组化检测IgG4表达与血清学检查一致。

Purpose To explore the clinicopathological features,diagnosis and differential diagnosis of autoimmune pancreatitis.Methods Clinical findings,morphologic features and immunohistochemical expression were investigated in 21 cases of autoimmune pancreatitis.Results 13 male patients and 8 female patients were included.The mean age was 58.8 years(ranged from 37 to 74 years).The main symptoms include jaundice in 10 patients and abdominal pain in 6 patients,while 5 patients were found by routine physical examination.18 patients were detected serum IgG4.Grossly,the lesion was a grey-white solid mass with a blunt border.Microscopically,chronic pancreatitis was found in the nodule of all cases,showing periductal lymphoplasmacytic infiltration with fibrosis.Phlebitis was seen in and around pancreas.More neutrophil infiltration with ductal epithelial destruction was found in 6 cases.Immunohistochemical stain of IgG4 was positive in 10 cases(IgG4+plasma cells>10/HPF),including 6 patients with elevated serum IgG4,2 without preoperative examination,and 2 with lightly elevated serum level of IgG4 but less than 2 times.Conclusion It is very difficult to differentiate mass-forming autoimmune pancreatitis from pancreatic cancer by clinical manifestation and image examination.Histopathology is the golden criteria with chronic lymphoplasmacytic sclerosing pancreatitis and venulitis,with/without granulocytic epithelial lesions.Immunohistochemical stain of IgG4 is consistant with serum examination.