Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka 589-8511, Japan; Mucosal Immunity Section, Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA.
Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, Osaka-Sayama, Osaka 589-8511, Japan.
Trends Immunol. 2018 Nov;39(11):874-889. doi: 10.1016/j.it.2018.09.005. Epub 2018 Oct 25.
Autoimmune pancreatitis (AIP) is a pancreatic manifestation of a recently defined disease form known as IgG4-related disease (AIP/IgG4-RD). AIP/IgG4-RD is characterized by elevated systemic IgG4 antibody concentrations and lesional tissues infiltrated by IgG4-expressing plasmacytes. In addition, recent studies have revealed that, in common with other autoimmune diseases, such as systemic lupus erythematosus (SLE) and psoriasis, AIP/IgG4-RD is associated with increased type I IFN (IFN-I) production by plasmacytoid dendritic cells (pDCs). However, unlike SLE, AIP/IgG4-RD is characterized by elevated IFN-I-dependent IL-33 production, the latter emerging as an important contributor to inflammation and fibrotic responses characterizing this disease. On this basis, we propose that blockade of the IFN-I/IL-33 axis might constitute a successful approach to treating this unique type of autoimmunity.
自身免疫性胰腺炎（AIP）是一种新近确定的疾病形式——IgG4 相关疾病（AIP/IgG4-RD）的胰腺表现。AIP/IgG4-RD 的特征是全身性 IgG4 抗体浓度升高，病变组织中 IgG4 表达浆细胞浸润。此外，最近的研究表明，与其他自身免疫性疾病（如系统性红斑狼疮[SLE]和银屑病）一样，AIP/IgG4-RD 与浆细胞样树突状细胞（pDC）产生的 I 型干扰素（IFN-I）增加有关。然而，与 SLE 不同，AIP/IgG4-RD 的特征是 IFN-I 依赖性 IL-33 产生增加，后者成为导致该疾病炎症和纤维化反应的重要因素。基于这一点，我们提出阻断 IFN-I/IL-33 轴可能是治疗这种独特类型自身免疫的一种成功方法。
