Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment

Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment

Blog Article

Cronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, Filing alopecia, and onychodystrophy.We report the case of a 45-year-old Caucasian male patient who failed multiple treatments over 2 years including steroids, azathioprine, adalimumab, and cyclosporine.He had recurrent and prolonged hospitalizations because of diarrhea, abdominal pain, weight loss, and malnutrition.Sirolimus was initiated with a significant clinical and endoscopic benefit apparent within, respectively, Underwear 2 and 8 weeks.An ongoing remission was achieved and maintained for over 6 months after prednisone tapering.

We review the current evidence on treatment of Cronkhite-Canada syndrome and suggest the incorporation of sirolimus in that algorithm.