Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Pancreas. 2011 Aug;40(6):809-14. doi: 10.1097/MPA.0b013e3182258a15.
The objective of this study was to clarify the clinical and pathophysiological characteristics of autoimmune pancreatitis (AIP) and its subtypes (lymphoplasmacytic sclerosing pancreatitis [LPSP] and idiopathic duct-centric pancreatitis [IDCP]) seen around the world.
An international multicenter survey of AIP was conducted in 15 institutes from 8 countries. We compared clinical and pathologic profiles of AIP (n = 731) and the clinical profiles of LPSP (n = 204) and IDCP (n = 64) patients.
Patients with LPSP were approximately 16 years older than IDCP patients. Obstructive jaundice was a more frequent presentation in LPSP versus IDCP (75% vs 47%, P < 0.001), whereas abdominal pain (41% vs 68%, P < 0.001) and acute pancreatitis (5% vs 34%, P < 0.001) were more frequent in IDCP patients. Patients with LPSP were more likely to have diffuse swelling of the pancreas (40% vs 25%, P = 0.037) and elevated serum IgG4 levels (63% vs 23%, P < 0.001) but less likely to be associated with ulcerative colitis (1% vs 16%, P < 0.001). Clinical profiles of non-histologically confirmed AIP from Asia, the United States, and United Kingdom corresponded with that of LPSP, whereas those from Italy and Germany suggested a mixture of LPSP and IDCP.
Autoimmune pancreatitis is seen all around the world, with regional differences in the pathologic and clinical features. Lymphoplasmacytic sclerosing pancreatitis and IDCP have distinct clinical profiles.
本研究旨在阐明世界各地自身免疫性胰腺炎（AIP）及其亚型（淋巴浆细胞硬化性胰腺炎[LPSP]和特发性胆管中心性胰腺炎[IDCP]）的临床和病理生理特征。
在来自 8 个国家的 15 个研究所进行了一项 AIP 的国际多中心调查。我们比较了 AIP（n=731）、LPSP（n=204）和 IDCP（n=64）患者的临床和病理特征。
LPSP 患者比 IDCP 患者年长约 16 岁。梗阻性黄疸在 LPSP 中比 IDCP 更常见（75%比 47%，P<0.001），而腹痛（41%比 68%，P<0.001）和急性胰腺炎（5%比 34%，P<0.001）在 IDCP 患者中更为常见。LPSP 患者更可能出现胰腺弥漫性肿胀（40%比 25%，P=0.037）和血清 IgG4 水平升高（63%比 23%，P<0.001），但与溃疡性结肠炎的关联较少（1%比 16%，P<0.001）。来自亚洲、美国和英国的非组织学确诊 AIP 的临床特征与 LPSP 相符，而来自意大利和德国的特征则提示 LPSP 和 IDCP 的混合存在。
AIP 遍布世界各地，其病理和临床特征存在区域性差异。LPSP 和 IDCP 具有不同的临床特征。
