Shrestha Pragya, Le Brian, Wagner Brent, Pompella William, Karmacharya Paras
Department of Medicine, Reading Hospital-Tower Health System, West Reading, PA 19611, USA.
Department of Pathology, Reading Hospital-Tower Health System, West Reading, PA 19611, USA.
Case Rep Rheumatol. 2018 Aug 23;2018:9602373. doi: 10.1155/2018/9602373. eCollection 2018.
IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.
IgG4相关性硬化性胆管炎（IgG4-SC）是IgG4相关性疾病（IgG4-RD）最常见的胰腺外表现之一，在临床上与原发性硬化性胆管炎（PSC）不同。IgG4-RD是一种越来越被认识的免疫介导的纤维炎性全身性疾病，主要影响中老年男性人群，可累及多个器官。如果存在IgG4-RD的胆管外临床表现，如腮腺和泪腺肿大、淋巴结病、自身免疫性胰腺炎和腹膜后纤维化等，则可为诊断提供重要线索。在这些特征的背景下，高血清IgG4水平、特征性影像学表现（如腊肠样胰腺或主动脉周炎）或活检结果（IgG4+浆细胞比例高、淋巴细胞浆细胞浸润、席纹状纤维化或闭塞性静脉炎）可诊断该病。然而，孤立性IgG4-SC可能是一个诊断挑战，而这种区分很重要，因为该疾病的治疗与其他胆管炎病因（如PSC）有很大不同。全身糖皮质激素治疗是主要的治疗方法。
