Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan.
Gut. 2013 Sep;62(9):1373-80. doi: 10.1136/gutjnl-2012-304224. Epub 2013 Jun 8.
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis characterised clinically by frequent presentation with obstructive jaundice, histologically by a lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to steroids. When so defined, AIP can be sub-classified into two subtypes, 1 and 2. Recent international consensus diagnostic criteria for AIP have been developed for diagnosis of both forms of AIP. Type 1 AIP is the pancreatic manifestation of a multiorgan disease, recently named IgG4-related disease. Little is known about the pathogenesis of either form of AIP. Despite frequent association of type 1 AIP with elevated serum IgG4 levels and infiltration with IgG4-positive plasma cells, it is unlikely that IgG4 plays a pathogenic role in AIP. Type 1 AIP responds to steroids, but there needs to be consensus on treatment regimens for induction and therapeutic end points. Relapses are common, but can be reduced by long-term use of low-dose steroids. Recent reports suggest that immunomodulators (azathioprine, 6-mercaptopurine and mycophenolate mofetil), as well biological agents (the antibody to CD20, rituximab) may have a role in maintaining remission in relapsing type 1 AIP. Future studies should clarify the best management options for treatment of relapses and maintenance of remission. Type 2 AIP is a pancreas-specific disorder not associated with IgG4. It presents in younger individuals equally with obstructive jaundice and pancreatitis. The inflammatory process responds to steroid therapy; relapses are uncommon. The clinical spectrum and long-term outcomes of medically treated type 2 AIP are still being evaluated.
自身免疫性胰腺炎（AIP）是一种慢性胰腺炎，其临床特征为频繁出现阻塞性黄疸，组织学表现为淋巴浆细胞浸润伴纤维化，治疗上对类固醇有显著反应。当如此定义时，AIP 可分为两个亚型，1 型和 2 型。最近为诊断这两种形式的 AIP 制定了国际共识的 AIP 诊断标准。1 型 AIP 是一种多器官疾病的胰腺表现，最近被命名为 IgG4 相关疾病。对于这两种形式的 AIP 的发病机制知之甚少。尽管 1 型 AIP 常与血清 IgG4 水平升高和 IgG4 阳性浆细胞浸润有关，但 IgG4 不太可能在 AIP 中发挥致病作用。1 型 AIP 对类固醇有反应，但需要就诱导和治疗终点的治疗方案达成共识。复发很常见，但长期使用低剂量类固醇可以减少复发。最近的报告表明，免疫调节剂（硫唑嘌呤、6-巯基嘌呤和霉酚酸酯）以及生物制剂（抗 CD20 抗体，利妥昔单抗）可能在维持复发性 1 型 AIP 的缓解中发挥作用。未来的研究应该阐明治疗复发和维持缓解的最佳管理方案。2 型 AIP 是一种与 IgG4 无关的胰腺特异性疾病。它在年轻患者中同样表现为阻塞性黄疸和胰腺炎。炎症反应对类固醇治疗有反应；复发少见。正在评估药物治疗的 2 型 AIP 的临床谱和长期结局。
