TY - JOUR A2 - Fan, Yu-Chen AU - Drazilova, Sylvia AU - Veseliny, Eduard AU - Lenartova, Patricia Denisa AU - Drazilova, Dagmar AU - Gazda, Jakub AU - Grgurevic, Ivica AU - Janicko, Martin AU - Jarcuska, Peter PY - 2021 DA - 2021/12/21 TI - IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease SP - 1959832 VL - 2021 AB - IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis.显示孔晶体素和微分诊断时,应区别于cholaniocarcivia或其他形式的colangitis(初级和二级colangitis)信息量增加和最近确定的诊断标准,IgG4相关割片 cholangitis在例行临床实践中仍然诊断不足诊断基础综合临床图片、实验室参数、语学发现和合影血清IgG4增量非专用性,但确实是日本Biltice协会建议诊断标准的一部分和IgG4-SCHisORt标准高血清IgG4保留临床实用性视高度大小而定约90%的病人同时有自免疫腹膜炎,10%的病人只与隔离收费约26%的病人有其他器官参与,如IgG4相关dapryadenitis/siladenitis、IgG4相关回发性纤维化或IgG4相关肾损伤以IgG4丰富淋巴素渗透为特征的全词学发现,消除单片膜炎和骨架纤维化实战难以捕捉,因为它从属定位组织学增益通过免疫史化学提高,并有IgG4阳性等离子细胞证据IgG-4相关割片合二为四子类型一线处理为cortcosteroids初始处理的目的是导出临床和实验室排解和合影规范30%的病人有复发课程,文献数据中,对慢性免疫抑制理疗没有共识疾病早期诊断后预知良好SN-2291-2789UR-https://doi.org/101155/2021/1959832DO-10.1155/2021/1959832JF-Canadacourse Enternology和HepatologyPB-HindawiKW-ER-ER