Sustained clinical response to infliximab in refractory Cronkhite-Canada syndrome

Abstract

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.

Cronkhite-Canada Syndrome: A Rare Cause of Gastrointestinal Polyposis With Response to Emerging Therapy

“…9 Moreover, reported cases have demonstrated some response to corticosteroid therapy or tumor necrosis factora inhibition. 7,[10][11][12][13][14] Several case reports include concurrent diagnoses of vitiligo, membranous nephropathy, hypothyroidism, and elevated antinuclear antibody titers. 6,[15][16][17][18] These comorbidities, serologic and histologic findings, and treatment responses are suggestive of a potential autoimmune mechanism underlying CCS.…”

Polyposis of gastrointestinal tract after COVID-19 mRNA vaccination: a report of two cases

“…However, corticosteroids, infliximab, azathioprine, and 5-aminosalicylate have been reported to have a positive effect in severe cases. 2 , 3 Gastrointestinal bleeding, malnutrition, and infection are major causes of death, and the patients are at substantial risk for gastrointestinal malignancies. 1 , 4 This paper presents two cases of Cronkhite-Canada syndrome occurring after COVID-19 mRNA vaccination.…”

CronkhiteCanada syndrome complicated with pulmonary embolism: A case report

“…One CCS patient with Hp infection was relieved after eradication of Hp and adjustment of intestinal flora[ 20 ]. The clinical symptoms of one patient with refractory CCS were completely relieved, and gastrointestinal polyps disappeared significantly after induction and maintenance treatment with infliximab[ 22 ]. Oral corticosteroids are the best treatment for CCS, providing relief of abdominal symptoms within a few months and causing polyps to disappear in over 6 months[ 23 ].…”