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Sustained clinical response to infliximab in refractory Cronkhite-Canada syndrome
  1. Caroline Di Jiang1,
  2. Helen Myint2,
  3. Andy Tie3 and
  4. Nigel H Stace4
  1. 1Gastroenterology, Wellington Hospital, Wellington, New Zealand
  2. 2Gastroenterology, Auckland City Hospital, Auckland, New Zealand
  3. 3Department of Pathology and Molecular Medicine, University of Otago, Wellington, New Zealand
  4. 4Department of Medicine, University of Otago, Wellington, New Zealand
  1. Correspondence to Dr Caroline Di Jiang; carolinedijiang{at}


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.

  • gastrointestinal system
  • drugs: gastrointestinal system
  • immunological products and vaccines

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  • Contributors The case report was written by CDJ and the pathology section by AT. This was subsequently reviewed by NHS and HM. The final submitted version was read and approved by all four authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.