Gastroenterology, Wellington Hospital, Wellington, New Zealand
Gastroenterology, Auckland City Hospital, Auckland, New Zealand.
BMJ Case Rep. 2020 Dec 21;13(12):e236990. doi: 10.1136/bcr-2020-236990.
A 59-year-old man with refractory Cronkhite-Canada syndrome (CCS) had poor clinical response to high-dose intravenous steroids, azathioprine, total parenteral nutrition and best supportive care. He remained highly symptomatic with abdominal pain, diarrhoea, recurrent sepsis and profound weight loss. Infliximab induction was given as rescue therapy, with marked clinical improvement observed within 3 weeks. This allowed steroid taper. Within 12 months of infliximab therapy, he achieved complete clinical remission and returned to his baseline weight and a full oral diet. Sequential endoscopies observed significant regression of previous marked gastrointestinal polyposis, including histological remission on colonic biopsies at 3.5 and 5 years of treatment. He currently remains in remission following 6 years of combination therapy with 5 mg/kg 8 weekly infliximab and azathioprine, and there is ongoing discussion with regard to the benefits and risks of therapy de-escalation. This case demonstrates the effectiveness of infliximab in inducing and maintaining remission in refractory CCS.
一位 59 岁的男性患有难治性 Cronkhite-Canada 综合征（CCS），对大剂量静脉注射类固醇、硫唑嘌呤、全胃肠外营养和最佳支持治疗反应不佳。他仍有严重的腹痛、腹泻、反复败血症和明显的体重减轻。给予英夫利昔单抗诱导治疗作为挽救性治疗，在 3 周内观察到明显的临床改善。这使得类固醇逐渐减量。在英夫利昔单抗治疗的 12 个月内，他达到了完全的临床缓解，恢复到基线体重和全口服饮食。连续内镜检查观察到先前明显的胃肠道息肉病有显著消退，包括在治疗 3.5 年和 5 年时结肠活检的组织学缓解。在接受每周 8 次 5mg/kg 的英夫利昔单抗和硫唑嘌呤联合治疗 6 年后，他目前仍处于缓解状态，并且正在讨论治疗降级的益处和风险。该病例表明英夫利昔单抗在诱导和维持难治性 CCS 缓解方面的有效性。
