Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan.
Dig Dis Sci. 2019 Sep;64(9):2385-2394. doi: 10.1007/s10620-019-05746-9.
Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic manifestation of IgG4-RD, and type 2 characterized by granulocytic epithelial lesions. The characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmocytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extrapancreatic manifestations of IgG4-RD (e.g., sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. These entities can be differentiated from mimickers by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line therapy is corticosteroids, or rituximab in high-risk patients with steroid intolerance. Although relapse rates are high, treatment of relapsed disease remains experimental.
自身免疫性胰腺炎（AIP）是自 1961 年以来被认识到的一种疾病。在发现升高的血清 IgG4 作为其诊断的有用生物标志物之前，Yoshida 博士于 1995 年首次描述了 AIP 实体，回想起来，它与目前 1 型 AIP 的概念非常相似。自 IgG4 被发现作为生物标志物（IgG4 时代）以来，一种新的 IgG4 相关疾病（IgG4-RD）的概念已被接受，它由两种亚型的 AIP 组成：1 型定义为 IgG4-RD 的胰腺表现，2 型的特征是粒细胞上皮病变。1 型 AIP 的特征是血清 IgG4 水平升高、淋巴浆细胞性硬化性胰腺炎（大量 IgG4+浆细胞和淋巴细胞浸润、席纹状纤维化和闭塞性静脉炎）、IgG4-RD 的胰外表现（如硬化性胆管炎、硬化性唾液腺炎、腹膜后纤维化）和对类固醇的反应性。这些疾病可以通过血清 IgG4 水平、影像学特征和组织病理学发现的组合与类似物区分开来。目前的一线治疗是类固醇，或对类固醇不耐受的高危患者使用利妥昔单抗。尽管复发率很高，但复发性疾病的治疗仍处于实验阶段。
