Zamboni Giuseppe, Lüttges Jutta, Capelli Paola, Frulloni Luca, Cavallini Giorgio, Pederzoli Paolo, Leins Alexander, Longnecker Daniel, Klöppel Günter
Department of Pathology, University of Verona, Verona, Italy.
Virchows Arch. 2004 Dec;445(6):552-63. doi: 10.1007/s00428-004-1140-z. Epub 2004 Oct 27.
Autoimmune pancreatitis seems to be a disease with a heterogeneous appearance. Our intention was to establish key diagnostic criteria, define grades of severity and activity, identify features of potential subtypes and evaluate the diagnostic relevance of biopsy specimens.
Histopathological criteria and clinical features were recorded in pancreatic resection specimens from 53 patients who were found to have chronic pancreatitis lacking pseudocysts, calculi, irregular duct dilatations, pancreas divisum and/or duodenal wall inflammation. The severity of the chronic inflammation was graded, and the activity of the acute inflammatory component and the granulocytic epithelial lesion (GEL) were determined. Additionally, pancreatic biopsy specimens from 9 patients with suspected AIP were assessed.
Periductal lymphoplasmacytic infiltration was identified in all cases, followed in order of frequency by periductal fibrosis and venulitis. These changes were absent in 147 pancreatic specimens that showed chronic pancreatitis associated with pseudocysts, calculi, pancreas divisum and/or duodenal wall inflammation. In 90% of the cases, these chronic changes were graded as 3 or 4. In 81%, the inflammatory process resided in the head of the pancreas and involved the common bile duct. GELs were present in 42% of the patients, who had a mean age of 40.5 years, an almost equal male-female ratio and a high coincidence of ulcerative colitis or Crohn's disease. Patients without GELs were older (mean age 64 years), showed a male preponderance, commonly had Sjogren's syndrome and often developed recurrent bile-duct stenosis. Diagnostically relevant lesions were present in two of five wedge biopsy specimens and three of four fine-needle specimens.
Periductal lymphoplasmacytic infiltration and fibrosis, preferential occurrence in the pancreatic head and venulitis characterize autoimmune pancreatitis. GELs predominantly occur in a subset of patients who are younger, more commonly have ulcerative colitis and Crohn's disease and seem to have fewer recurrences than patients without GELs. Pancreatic biopsy material proved to be a very helpful adjunct for establishing the diagnosis.
自身免疫性胰腺炎似乎是一种表现具有异质性的疾病。我们的目的是确立关键诊断标准，界定严重程度和活动度分级，识别潜在亚型的特征，并评估活检标本的诊断相关性。
记录了53例慢性胰腺炎患者胰腺切除标本的组织病理学标准和临床特征，这些患者不存在假性囊肿、结石、不规则导管扩张、胰腺分裂症和/或十二指肠壁炎症。对慢性炎症的严重程度进行分级，并确定急性炎症成分和粒细胞上皮病变（GEL）的活动度。此外，对9例疑似自身免疫性胰腺炎患者的胰腺活检标本进行了评估。
所有病例均发现导管周围淋巴浆细胞浸润，其次按频率依次为导管周围纤维化和静脉炎。在147例显示与假性囊肿、结石、胰腺分裂症和/或十二指肠壁炎症相关的慢性胰腺炎的胰腺标本中未发现这些改变。90%的病例中，这些慢性改变被评为3级或4级。81%的炎症过程位于胰头并累及胆总管。42%的患者存在GEL，这些患者的平均年龄为40.5岁，男女比例几乎相等，溃疡性结肠炎或克罗恩病的并发率较高。无GEL的患者年龄较大（平均年龄64岁），男性占优势，常见干燥综合征，且常发生复发性胆管狭窄。5份楔形活检标本中有2份以及4份细针穿刺标本中有3份存在诊断相关病变。
导管周围淋巴浆细胞浸润和纤维化、在胰头的优先发生以及静脉炎是自身免疫性胰腺炎的特征。GEL主要发生在一部分较年轻的患者中，这些患者更常见溃疡性结肠炎和克罗恩病，且似乎比无GEL的患者复发次数更少。胰腺活检材料被证明是确立诊断的非常有用的辅助手段。
