Gonzalez Hector H, Skrove Jamie L, Rosen Seth, Sobrado Javier
Internal Medicine, Florida Atlantic University Charles E. Schmidt College of Medicine, Boca Raton, USA.
Internal Medicine, Larkin Community Hospital, South Miami, USA.
Cureus. 2019 Feb 28;11(2):e4153. doi: 10.7759/cureus.4153.
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a novel entity that belongs to the immune-mediated fibroinflammatory class of IgG4-related diseases (IgG4-RD). IgG4-SC is noted to be one of the most frequent manifestations of extra-pancreatic disease among IgG4-RD, which is significantly different from primary SC (PSC) and cholangiocarcinoma (CC) as is evident in the varied approaches to treatment. IgG4-RD includes IgG4-SC and autoimmune pancreatitis (AIP). Herein, we presented a case of IgG4-SC in a patient with obstructive jaundice secondary to AIP. We have also discussed the current recommendations for diagnostic and treatment modalities, with an emphasis on the issues that arise in obtaining a definitive classification of disease.
免疫球蛋白G4相关性硬化性胆管炎(IgG4-SC)是一种新型疾病,属于免疫介导的IgG4相关性疾病(IgG4-RD)的纤维炎性类别。IgG4-SC是IgG4-RD中最常见的胰腺外疾病表现之一,与原发性硬化性胆管炎(PSC)和胆管癌(CC)有显著差异,这在不同的治疗方法中很明显。IgG4-RD包括IgG4-SC和自身免疫性胰腺炎(AIP)。在此,我们报告了一例继发于AIP的梗阻性黄疸患者的IgG4-SC病例。我们还讨论了目前关于诊断和治疗方式的建议,重点是在获得疾病明确分类时出现的问题。