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维多珠单抗治疗与常见变异型免疫缺陷相关的难治性肠病

Vedolizumab Therapy in Refractory Enteropathy Associated With CVID.

作者信息

Johnson Dane, Lee Goo, Weber Frederick

机构信息

Division of Gastroenterology and Hepatology, University of Alabama at Birmingham, Birmingham, Alabama.

Department of Pathology, University of Alabama at Birmingham, Birmingham, Alabama.

出版信息

ACG Case Rep J. 2021 Dec 28;9(1):e00721. doi: 10.14309/crj.0000000000000721. eCollection 2022 Jan.

Abstract

Common variable immunodeficiency (CVID) is a disorder of typically adult-onset hypogammaglobulinemia in the absence of other known causes. Noninfectious gastrointestinal tract manifestations are common, and a subset of patients with CVID present with a severe enteropathy manifesting as severe malabsorption, weight loss, and diarrhea. A 63-year-old man presented with CVID and severe enteropathy who failed to improve with cytomegalovirus therapy, multiple empiric therapies, and corticosteroids. Vedolizumab infusions were initiated with a dramatic, rapid, and durable complete clinical response.

摘要

普通可变免疫缺陷(CVID)是一种通常在成人期发病的低丙种球蛋白血症疾病,不存在其他已知病因。非感染性胃肠道表现很常见,一部分CVID患者会出现严重肠病,表现为严重吸收不良、体重减轻和腹泻。一名63岁男性患有CVID和严重肠病,接受巨细胞病毒治疗、多种经验性治疗和皮质类固醇治疗均未见改善。开始使用维多珠单抗输注后,患者出现了显著、快速且持久的完全临床缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/90ae/8716098/7bc6f67ee9c3/ac9-9-e00721-g001.jpg

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