自身免疫性胰腺炎的临床和病理特征

Clinical and pathological features of autoimmune pancreatitis

自身免疫性胰腺炎(autoimmune pancreatitis,AIP)是一种罕见的具有独特的临床和组织形态学特点、以自身免疫性为病因的慢性胰腺炎。由于AIP患者的其他器官或部位常有相似的组织学改变,且都有免疫球蛋白G4(immunoglobin G4,IgG4)阳性浆细胞组织内浸润增多,以及对类固醇激素治疗有效,由此提出了一种全新的与IgG4相关的全身性疾病(IgG4-associated systemic disease,ISD)的临床病理概念。本文主要阐述AIP及ISD的临床表现、诊断标准、病理学特点及其相关的胰腺外器官表现。

Autoimmune pancreatitis（AIP） is a rare disorder of autoimmune etiology that is associated with special clinical,histological,and morphologic features.Because AIP patients show similar histological changes in different organs or tissues,such as the increased immunoglobin G4（IgG4）-positive cell infiltrate and therapeutic effect to steroid treatment,a new clinicopathological concept of IgG4-related systemic disease（ISD） has been proposed.In this review,we will discuss the clinical and histological features,diagnostic criteria in AIP and ISD.The associated extrapancreatic manifestations are also discussed.