Nakazawa Takahiro, Shimizu Shuya, Naitoh Itaru
Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan.
Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
Semin Liver Dis. 2016 Aug;36(3):216-28. doi: 10.1055/s-0036-1584321. Epub 2016 Jul 28.
More men than women develop immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Age at clinical onset is significantly older in patients with IgG4-SC. Patients with IgG4-SC appear similar to those with cholangiocarcinoma and primary sclerosing cholangitis (PSC). The association between IgG4-SC and autoimmune pancreatitis (AIP) is useful for the diagnosis of IgG4-SC. However, some IgG4-SC cases are isolated from AIP and are difficult to diagnose. The authors focus on three distinct features of IgG4-SC. First, diffuse inflammation induces a longer stenosis on cholangiography in contrast to the short stenosis of patients with PSC. Second, fibroinflammatory involvement is observed mainly in the stroma of the bile duct wall, whereas the bile duct epithelium is intact. Third, steroid therapy results in remarkable improvement. Although the prognosis of patients with IgG4-SC is good, some cases have developed portal hypertension and liver cirrhosis during their clinical course. Further study is needed to elucidate the long-term outcomes and mechanism of IgG4-SC.
患免疫球蛋白G4相关性硬化性胆管炎（IgG4-SC）的男性多于女性。IgG4-SC患者临床发病年龄显著偏大。IgG4-SC患者与胆管癌和原发性硬化性胆管炎（PSC）患者表现相似。IgG4-SC与自身免疫性胰腺炎（AIP）之间的关联有助于IgG4-SC的诊断。然而，一些IgG4-SC病例与AIP无关，难以诊断。作者关注IgG4-SC的三个不同特征。第一，与PSC患者的短狭窄不同，弥漫性炎症在胆管造影中导致更长的狭窄。第二，纤维炎症主要累及胆管壁的基质，而胆管上皮完整。第三，类固醇治疗可带来显著改善。尽管IgG4-SC患者的预后良好，但一些病例在临床过程中发展为门静脉高压和肝硬化。需要进一步研究以阐明IgG4-SC的长期结局和机制。
