S3885 Vedolizumab as an Effective Therapy in the Treatment of Cronkhite-Canada Syndrome Refractory to Standard Medical Therapy: A Case Report

Introduction: Cronkhite-Canada Syndrome (CCS) is a rare, non-hereditary gastrointestinal disorder marked by diffuse polyposis, diarrhea, alopecia, skin pigmentation, and nail atrophy. Its cause is unclear, though an autoimmune etiology is suspected. Complications include malnutrition, protein-losing enteropathy, and increased cancer risk. Treatment typically involves immunosuppressive therapy with corticosteroids, azathioprine, or infliximab. Vedolizumab, a gut-selective monoclonal antibody that blocks lymphocyte migration, may offer targeted immunosuppression. This case highlights its successful use in maintaining stable polyposis in CCS and treating CCS-associated malnutrition unresponsive to standard therapies. Case Description/Methods: A 51-year-old man underwent surveillance colonoscopy, which revealed numerous polyps; biopsies confirmed a diagnosis of CCS. His symptoms included chronic diarrhea, hair loss, and unintentional weight loss. Initial treatment with prednisone and azathioprine was initiated, but azathioprine was discontinued due to worsening anemia. The patient subsequently developed thromboembolic disease complicated by intestinal ischemia and perforation, requiring small bowel resection and creation of an end ileostomy. His recovery was further complicated by high ileostomy output and membranous glomerulonephritis, necessitating prolonged corticosteroid therapy. Following prednisone taper, infliximab was administered for 1 year without improvement in high ileostomy output or hypoalbuminemia. Moreover, a follow-up colonoscopy revealed over 100 colonic polyps, prompting ileostomy takedown, partial colectomy, and consideration of alternative therapy. Vedolizumab was then initiated, leading to sustained improvement in both diarrhea and hypoalbuminemia. The patient declined total abdominal colectomy and instead elected for surveillance endoscopies every 6 months, which have shown stable polyposis over 6 years since starting vedolizumab. Discussion: This case highlights the potential efficacy of vedolizumab as a therapeutic option for CCS, particularly in patients unresponsive to conventional treatments. Given the rarity of CCS, randomized controlled trials are not feasible, and treatment decisions often rely on limited case reports, extrapolated data, and clinical judgment. Successful outcomes, as demonstrated here, underscore the importance of documenting and sharing such experiences to help inform future management strategies and improve patient care.