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

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

Cronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, alopecia, and onychodystrophy. We report the case of a 45-year-old Caucasian male patient who failed multiple treatments over 2 years includin...

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Main Authors: Catherine Langevin MD, Hugo Chapdelaine MD, Jean-Maxime Picard MD, Pierre Poitras MD, Raymond Leduc MD, FRCPC
Format: Article
Language:English
Published: SAGE Publishing 2018-03-01
Series:Journal of Investigative Medicine High Impact Case Reports
Online Access:https://doi.org/10.1177/2324709618765893
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spelling doaj-0015f47bd7f04f82ae63c7032687c10f2020-11-25T03:34:11ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962018-03-01610.1177/2324709618765893Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard TreatmentCatherine Langevin MD0Hugo Chapdelaine MD1Jean-Maxime Picard MD2Pierre Poitras MD3Raymond Leduc MD, FRCPC4Centre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaMontreal Clinical Research Institute, Montreal, Quebec, CanadaChicoutimi Hospital, Chicoutimi, Quebec, CanadaCentre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaCentre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaCronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, 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, 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.https://doi.org/10.1177/2324709618765893
collection DOAJ
language English
format Article
sources DOAJ
author Catherine Langevin MD
Hugo Chapdelaine MD
Jean-Maxime Picard MD
Pierre Poitras MD
Raymond Leduc MD, FRCPC
spellingShingle Catherine Langevin MD
Hugo Chapdelaine MD
Jean-Maxime Picard MD
Pierre Poitras MD
Raymond Leduc MD, FRCPC
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
Journal of Investigative Medicine High Impact Case Reports
author_facet Catherine Langevin MD
Hugo Chapdelaine MD
Jean-Maxime Picard MD
Pierre Poitras MD
Raymond Leduc MD, FRCPC
author_sort Catherine Langevin MD
title Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
title_short Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
title_full Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
title_fullStr Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
title_full_unstemmed Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
title_sort sirolimus in refractory cronkhite-canada syndrome and focus on standard treatment
publisher SAGE Publishing
series Journal of Investigative Medicine High Impact Case Reports
issn 2324-7096
publishDate 2018-03-01
description Cronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, 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, 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.
url https://doi.org/10.1177/2324709618765893
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