Ⅰ型自身免疫性胰腺炎的研究进展

任崧; 叶兆祥; 朱理珉

doi:10.3969/j.issn.1001-5256.2014.02.023

Ⅰ型自身免疫性胰腺炎的研究进展

DOI: 10.3969/j.issn.1001-5256.2014.02.023

天津市肿瘤防治重点实验室,国家肿瘤临床医学研究中心,天津医科大学肿瘤医院放射科

详细信息

中图分类号: R576
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出版历程
- 收稿日期: 2013-05-30
- 出版日期: 2014-02-20

Research progress in type I autoimmune pancreatitis

Key Laboratory of Cancer Prevention and Therapy, Department of Radiology, Tianjin Medical University Cancer Institute and Hospital

摘要

摘要: 自身免疫性胰腺炎（AIP）是逐渐被认识的慢性胰腺炎的一种类型,与其他类型的慢性胰腺炎有明显的不同。随着2001年发现AIP患者血清IgG4含量明显增高,这个现实被广泛的接受。1型AIP与2型AIP不同。2%的慢性胰腺炎为1-AIP,成人男性患者居多。患者通常由于胰头的增大或胆管壁增厚所致梗阻性黄疸,通过血清学、影像学和组织学与胰腺癌鉴别。血清IgG4水平的增高是最为敏感和特异性的表现。影像学的表现为胰管的不规则狭窄,胰腺弥散性或局灶性的扩大及胰周可见环形的囊壳样影,以及影像延迟期强化。组织学显示大量的小叶间质表现为致密的纤维化伴席纹状、炎细胞浸润,闭塞性静脉炎和IgG4阳性细胞数量增加。激素治疗疗效明显是另外的特征,在初治的23周血清学和影像学明显改善。基于同步或不同步的伴有多器官的损伤,1-AIP也被认为是一种IgG4相关全身性疾病。至今有一些与1-AIP相关的自身免疫性抗原被鉴定,但IgG4在此病中的作用是肯定的。
- 胰腺炎,慢性 /
- 自身免疫疾病 /
- 受体,IgG /
- 综述
Abstract: Autoimmune pancreatitis ( AIP) is an increasingly recognized type of chronic pancreatitis, which is clearly distinct from other types of chronic pancreatitis. After it was discovered in 2001 that serum IgG4 concentrations are significantly elevated in AIP patients, the distinctiveness of this disease has been widely recognized. Type 1 AIP, unlike type 2 AIP, accounts for 2% of chronic pancreatitis cases, and it predominantly affects adult males. Patients usually present with obstructive jaundice due to enlargement of the pancreatic head or thickening of the lower bile duct wall. Pancreatic cancer is the leading differential diagnosis for which serological, imaging, and histological examinations need to be considered. Serologically, an elevated level of IgG4 is the most sensitive and specific finding for AIP. Imaging features include irregular narrowing of the pancreatic duct, diffuse or focal enlargement of the pancreas, capsule- like peri- pancreatic rim, and enhancement at the late phase of contrast- enhanced images. Biopsy or surgical specimens show much of the intralobular interstitial tissue has diffuse lymphoplasmacytic infiltration containing many IgG4- positive plasma cells, storiform fibrosis, and obliterative phlebitis. A dramatic response to steroid therapy is another characteristic, and serological or radiological improvements are normally identified within the first 2 or 3 weeks. Type 1 AIP is estimated as a pancreatic manifestation of a systemic IgG4- related disease based on the fact that synchronous or metachronous lesions can develop in multiple organs. Several autoantigens related to type 1 AIP have been identified so far, but the exact role of IgG4 in this disease remains to be clarified.
- pancreatitis, chronic /
- autoimmune diseases /
- receptors, IgG /
- review

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