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自身免疫性胰腺炎

Author
Norton J Greenberger, MD
Section Editor
David C Whitcomb, MD, PhD
Deputy Editor
Shilpa Grover, MD, MPH, AGAF
Translators
汤丽平, 副主任医师，副教授

引言

自身免疫性胰腺炎(autoimmune pancreatitis, AIP)是一种很少被识别的(推测的)自身免疫性病因所致的疾病，具有特征性临床、组织学及形态学表现[1]。有关AIP的早期文献大部分来自于当地AIP发病率不断上升(可能因为对AIP的识别提高)的日本[2]。然而，该病已在欧洲多个国家、美国及韩国等地被发现，表明AIP其实是一种遍布全世界的疾病[3]。

AIP可以作为原发性胰腺疾病而发生，也可与其他推测由自身免疫性病因所致疾病有关，包括免疫球蛋白G4(immunoglobulin G4, IgG4)相关性胆管炎、唾液腺疾病、纵膈纤维化、腹膜后纤维化、肾小管间质性疾病以及炎症性肠病等(表 1)。一些已发表的资料指出，AIP实际上是一种与IgG4相关的系统性疾病[4,5]。 (参见“IgG4-相关性疾病的概述”)

相关术语

部分根据AIP特定的病理学表现及胰腺外临床表现的存在，它有若干个名称，包括硬化性胰腺炎、肿瘤样胰腺炎及非酒精性阻塞性胰腺炎[6,7]。然而，目前普遍认为其病理异质性可能反映了同一疾病的不同分期或不同临床表现[6,7]。

对于AIP患者而言，IgG4阳性浆细胞被认为是该病的标志，且能在胰腺及其他多种组织中检测到[8-12]。此外，大部分患者的血清IgG4水平升高至正常上限的2倍以上。 (参见下文‘其他临床表现’和‘IgG4血清学检查’)

尽管较早期的报道将AIP与原发性硬化性胆管炎(primary sclerosing cholangitis, PSC)联系起来，但目前已经认识到肝内胆管和肝外胆管畸形伴狭窄最常见的原因是IgG4相关性胆管炎。研究已发现临床表现提示PSC的患者有9%-36%出现IgG4水平升高，而在其他慢性肝脏疾病患者中，该数值仅为1%[13]。 (参见下文‘鉴别IAC和PSC’)

                 

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Literature review current through: 2017-06 . | This topic last updated: 2015-12-22.
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