Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota 55901, USA.
Curr Opin Gastroenterol. 2010 Sep;26(5):513-8. doi: 10.1097/MOG.0b013e32833d118b.
To summarize the existing knowledge of autoimmune pancreatitis (AIP) and to review the progress made in the diagnosis and treatment of AIP in the past year.
The term 'AIP' appears to encompass at least two distinct subtypes, type 1 and type 2. Type 1 AIP is the pancreatic manifestation of a systemic fibroinflammatory disease called immunoglobulin G4-associated systemic disease. Type 2 AIP affects younger patients, does not have a gender predilection and is associated with normal serum immunoglobulin G4 levels. Existing criteria are geared toward diagnosis of type 1; type 2 AIP can be definitively diagnosed only on pancreatic histology. Both subtypes respond to corticosteroid therapy. However, there are no standardized protocols for initial treatment or management and prevention of relapses in AIP. A novel antibody for AIP has recently been identified and its performance needs validation from other centers. Newly published strategies for differentiating AIP from pancreatic cancer are available.
AIP is a rare disease whose recognition and understanding are evolving. Much needs to be elucidated with regard to its cause, pathogenesis, treatment of relapse and long-term outcomes. A multidisciplinary team, familiar with the disease, is critical in making the correct diagnosis.
总结自身免疫性胰腺炎(AIP)的现有知识,并复习过去一年中 AIP 的诊断和治疗进展。
“AIP”一词似乎至少包含两种不同的亚型,即 1 型和 2 型。1 型 AIP 是一种称为 IgG4 相关系统性疾病的全身性纤维炎症性疾病的胰腺表现。2 型 AIP 影响年轻患者,无性别倾向,且与正常血清 IgG4 水平相关。现有的标准旨在诊断 1 型;2 型 AIP 只能通过胰腺组织学来明确诊断。两种亚型均对皮质类固醇治疗有反应。然而,目前尚无 AIP 初始治疗或管理以及预防复发的标准化方案。最近发现了一种针对 AIP 的新型抗体,其性能需要其他中心进行验证。目前已有可用于区分 AIP 和胰腺癌的新策略。
AIP 是一种罕见疾病,其识别和理解仍在不断发展。需要阐明其病因、发病机制、复发的治疗和长期预后等诸多问题。熟悉该疾病的多学科团队对于做出正确诊断至关重要。