一例表现为炎性假瘤和硬化性胆管炎的肝脏免疫球蛋白G4相关性疾病病例。
A Case of Hepatic Immunoglobulin G4-Related Disease Presenting as an Inflammatory Pseudotumor and Sclerosing Cholangitis.
作者信息
Jang Se Young, Han Young Seok, Lee Sang Yub, Han Ja Ryung, Kweon Young Oh, Tak Won Young, Park Soo Young, Lee Yu Rim, Ryeom Hun Kyu, Cha Jung Guen, Hong Jihoon, Kang Yoo Na
机构信息
Department of Internal Medicine, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, 130 Dongdeok-ro, Jung-gu, Daegu 41944, Korea.
Department of Surgery, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, 130 Dongdeok-ro, Jung-gu, Daegu 41944, Korea.
出版信息
Diagnostics (Basel). 2022 Jun 19;12(6):1497. doi: 10.3390/diagnostics12061497.
An inflammatory pseudotumor is a benign disease characterized by tumor-like lesions consisting of inflammatory cells including plasma cells and fibrous tissue. Recently, some inflammatory pseudotumor cases proved to be a form of Immunoglobulin G4-related disease (IgG4-RD). This novel clinical entity, recognized as a fibroinflammatory condition, is characterized by lymphoplasmacytic infiltration with a predominance of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We report a case of IgG4-RD in the form of an inflammatory pseudotumor in the liver with combined sclerosing cholangitis. We recommend that for diagnosing IgG4-RD accurately, it is important to obtain adequate tissue samples and follow-up the lesion in clinical practice.
炎性假瘤是一种良性疾病，其特征为肿瘤样病变，由包括浆细胞在内的炎性细胞和纤维组织构成。最近，一些炎性假瘤病例被证实为免疫球蛋白G4相关疾病（IgG4-RD）的一种形式。这种新确认的临床实体被认为是一种纤维炎性病症，其特征为淋巴细胞和浆细胞浸润，以IgG4阳性浆细胞为主，呈席纹状纤维化，且血清IgG4浓度常升高。我们报告1例表现为肝脏炎性假瘤合并硬化性胆管炎的IgG4-RD病例。我们建议，在临床实践中，为准确诊断IgG4-RD，获取足够的组织样本并对病变进行随访很重要。
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