TY - JOUR A2 - Handra-Luca, Adriana AU - Thinesen, Malene Theilmann AU - Schaffalitzky de Muckadell, Ove B. AU - Detlefsen，Sönke PY - 2018 DA - 2018/09/16 TI - igg4相关硬化性胆管炎伴肝内胆管活检诊断SP - 2309293 VL - 2018 AB - igg4相关疾病以淋巴浆细胞炎症和纤维化为特征，通常导致不同器官的肿块形成病变。igg4相关疾病影响胆管称为igg4相关硬化性胆管炎。74岁男性主诉吞咽困难和腹痛。内镜逆行胆管造影和磁共振胆管造影发现胆管病变，怀疑胆管癌或胆管炎。肝活检显示层状纤维化，淋巴浆细胞浸润，闭塞性静脉炎，门脉扩张的门脉炎性结节。热点显示每高能场(HPF)有339个IgG4阳性细胞，IgG4/IgG比值为72%。8个月前，腹股沟淋巴结被切除，显示滤泡间区扩大，浆细胞增多。热点显示IgG4阳性细胞593个，IgG4/IgG比值为92%。患者血清IgG4升高近正常上限10倍。 The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was good response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease. SN - 2090-6781 UR - https://doi.org/10.1155/2018/2309293 DO - 10.1155/2018/2309293 JF - Case Reports in Pathology PB - Hindawi KW - ER -