IgG4-Sclerosing Cholangitis in a Pediatric Patient
IgG4-Sclerosing Cholangitis in a Pediatric Patient
复制标题
儿科患者的 IgG4 硬化性胆管炎
DOI：
10.1055/s-0034-1398475
复制
发表时间：
2015
影响因子：
4.2
通讯作者：
Jaime Chu
中科院分区：
文献类型：
--
作者：
Danya Rosen;S. Thung;Shari Sheflin;Joanne Lai;Ally Rosen;R. Arnon;Jaime Chu
摘要IgG 4硬化性胆管炎（IgG 4-SC）是一种免疫介导的过程，导致胰胆管的炎症和纤维化。尽管IgG 4-SC主要与自身免疫性胰腺炎相关，但IgG 4-SC作为其自身实体可能难以诊断。IgG 4-SC患者通常为60岁以上的男性，临床表现为梗阻性黄疸、腹痛和体重减轻。IgG 4-SC的诊断可能难以与原发性硬化性胆管炎（PSC）或胆管癌鉴别。IgG 4-SC的形态学特征为致密淋巴浆细胞浸润，特别是IgG 4+浆细胞和CD 4 + T细胞，胆管壁广泛纤维化和闭塞性静脉炎。与PSC相反，IgG 4-SC患者的血清IgG 4水平通常升高，可以成功地用免疫抑制治疗。在这里，我们提出了第一个报告的情况下，IgG 4-SC的儿科患者无症状的肝酶升高，胆管狭窄的成像，特征性病理结果，血清IgG 4升高，和良好的反应，皮质类固醇。儿科胃肠病学家和肝病学家以及儿科肝脏病理学家需要了解IgG 4-SC作为一种疾病实体。尽管某些临床和影像学表现与PSC相似，但IgG 4-SC的诊断及其适当的皮质类固醇治疗通常会导致疾病的缓解和逆转。
Abstract IgG4 sclerosing cholangitis (IgG4-SC) is an immune-mediated process that results in inflammation and fibrosis of the pancreatobiliary tract. Although IgG4-SC is predominantly associated with autoimmune pancreatitis, IgG4-SC as its own entity can be difficult to diagnose. Patients with IgG4-SC are typically men over the age of 60, and present clinically with obstructive jaundice, abdominal pain, and weight loss. The diagnosis of IgG4-SC may be difficult to differentiate from primary sclerosing cholangitis (PSC) or cholangiocarcinoma. IgG4-SC is morphologically characterized by dense lymphoplasmacellular infiltration, particularly IgG4+ plasma cells and CD4+ T cells, extensive fibrosis in bile duct walls, and obliterative phlebitis. In contrast to PSC, those with IgG4-SC often have elevated serum IgG4 and can be successfully treated with immunosuppression. Here, we present the first reported case of IgG4-SC in a pediatric patient with asymptomatic elevation in liver enzymes, bile duct strictures on imaging, characteristic pathology findings, elevated serum IgG4, and excellent response to corticosteroids. Pediatric gastroenterologists and hepatologists, as well as pediatric hepatopathologists, need to be aware of IgG4-SC as a disease entity. Although certain clinical and imaging findings mimic PSC, diagnosis of IgG4-SC and its appropriate treatment with corticosteroids often lead to remission and reversal of disease.
影响因子：
3.8
作者：
Ferrone, Cristina R.;Pieretti-Vanmarcke, Rafael;Bloom, Jordan P.;Zheng, Hui;Szymonifka, Jackye;Wargo, Jennifer A.;Thayer, Sarah P.;Lauwers, Gregory Y.;Deshpande, Vikram;Mino-Kenudson, Mari;Fernandez-del Castillo, Carlos;Lillemoe, Keith D.;Warshaw, Andrew L.
通讯作者：
Warshaw, Andrew L.