英夫利昔单抗成功治疗模仿结肠克罗恩病的慢性肉芽肿病
Chronic Granulomatous Disease Mimicking Colonic Crohn's Disease Successfully Treated with Infliximab.
作者信息
Peixoto Armando, Coelho Rosa, Maia Tiago, Sarmento António, Magro Fernando, Macedo Guilherme
机构信息
Department of Gastroenterology, Centro Hospitalar São João, Porto, Portugal; WGO Porto Training Center, Porto, Portugal.
Department of Pathology, Centro Hospitalar de São João, Porto, Portugal.
出版信息
ACG Case Rep J. 2017 Mar 29;4:e46. doi: 10.14309/crj.2017.46. eCollection 2017.
Chronic granulomatous disease (CGD) is a genetically induced disease caused by mutations in one of the components of the NADPH-oxidase in phagocytes, characterized by life-threatening bacterial and fungal infections and granuloma formation. Treatment includes prevention of infectious complications and immunomodulation. However, a standard strategy is not yet defined. The authors report an X-linked CGD female carrier who presented during adulthood with diarrhea and colorectal ulcers, with high impairment of quality of life. Induction with infliximab 5 mg/kg (weeks 0, 2, and 6) with infectious prophylaxis was initiated. She continued infliximab 5 mg/kg every 8 weeks with complete symptomatic response at 15 months.
慢性肉芽肿病（CGD）是一种由吞噬细胞中烟酰胺腺嘌呤二核苷酸磷酸（NADPH）氧化酶的一种成分发生突变引起的遗传性疾病，其特征为危及生命的细菌和真菌感染以及肉芽肿形成。治疗包括预防感染并发症和免疫调节。然而，尚未确定标准策略。作者报告了一名成年期出现腹泻和结肠直肠溃疡、生活质量严重受损的X连锁CGD女性携带者。开始使用英夫利昔单抗5 mg/kg（第0、2和6周）诱导治疗并进行感染预防。她每8周继续使用英夫利昔单抗5 mg/kg，15个月时症状完全缓解。
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