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Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports

BACKGROUND: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disorder, characterized by gastrointestinal polyposis and ectodermal changes. The pathophysiology remains unclear. Treatment with corticosteroids is considered the mainstay treatment because of its high efficacy. However, some patie...

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Autores principales: Yamakawa, Kohei, Yoshino, Takuya, Watanabe, Kotaro, Kawano, Koichiro, Kurita, Akira, Matsuzaki, Naomi, Yuba, Yoshiaki, Yazumi, Shujiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5053148/
https://www.ncbi.nlm.nih.gov/pubmed/27716071
http://dx.doi.org/10.1186/s12876-016-0541-1
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author Yamakawa, Kohei
Yoshino, Takuya
Watanabe, Kotaro
Kawano, Koichiro
Kurita, Akira
Matsuzaki, Naomi
Yuba, Yoshiaki
Yazumi, Shujiro
author_facet Yamakawa, Kohei
Yoshino, Takuya
Watanabe, Kotaro
Kawano, Koichiro
Kurita, Akira
Matsuzaki, Naomi
Yuba, Yoshiaki
Yazumi, Shujiro
author_sort Yamakawa, Kohei
collection PubMed
description BACKGROUND: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disorder, characterized by gastrointestinal polyposis and ectodermal changes. The pathophysiology remains unclear. Treatment with corticosteroids is considered the mainstay treatment because of its high efficacy. However, some patients have steroid-resistant CCS. The therapeutic strategy for steroid-resistant CCS is not yet established. We report two cases with steroid-resistant CCS that were effectively treated with cyclosporine (CyA). We evaluated the therapeutic strategy for steroid-resistant CCS based on reviews of previous reports. CASE PRESENTATION: Our patients with CCS were first treated with prednisolone. No clinical response was noted, and treatment with CyA was initiated. After beginning CyA treatment, both clinical symptoms and polyposis markedly improved. Up to the present, 55 cases of CCS treated with corticosteroids and their response were reported. Out of the 57 patients, including our 2 cases, 9 (16 %) did not respond clinically to corticosteroids. In 7 of the 9 steroid-resistant cases, the prognosis after corticosteroids treatment was described. In 5 of the 7 steroid-resistant cases, immunosuppressive treatments induced remission. In 4 of these 5 cases, moreover, the key drug of treatments was calcineurin inhibitor. CONCLUSIONS: Treatment with calcineurin inhibitor, such as CyA, could be a potential option for steroid-resistant CCS. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12876-016-0541-1) contains supplementary material, which is available to authorized users.
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spelling pubmed-50531482016-10-06 Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports Yamakawa, Kohei Yoshino, Takuya Watanabe, Kotaro Kawano, Koichiro Kurita, Akira Matsuzaki, Naomi Yuba, Yoshiaki Yazumi, Shujiro BMC Gastroenterol Case Report BACKGROUND: Cronkhite-Canada syndrome (CCS) is a rare non-inherited disorder, characterized by gastrointestinal polyposis and ectodermal changes. The pathophysiology remains unclear. Treatment with corticosteroids is considered the mainstay treatment because of its high efficacy. However, some patients have steroid-resistant CCS. The therapeutic strategy for steroid-resistant CCS is not yet established. We report two cases with steroid-resistant CCS that were effectively treated with cyclosporine (CyA). We evaluated the therapeutic strategy for steroid-resistant CCS based on reviews of previous reports. CASE PRESENTATION: Our patients with CCS were first treated with prednisolone. No clinical response was noted, and treatment with CyA was initiated. After beginning CyA treatment, both clinical symptoms and polyposis markedly improved. Up to the present, 55 cases of CCS treated with corticosteroids and their response were reported. Out of the 57 patients, including our 2 cases, 9 (16 %) did not respond clinically to corticosteroids. In 7 of the 9 steroid-resistant cases, the prognosis after corticosteroids treatment was described. In 5 of the 7 steroid-resistant cases, immunosuppressive treatments induced remission. In 4 of these 5 cases, moreover, the key drug of treatments was calcineurin inhibitor. CONCLUSIONS: Treatment with calcineurin inhibitor, such as CyA, could be a potential option for steroid-resistant CCS. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12876-016-0541-1) contains supplementary material, which is available to authorized users. BioMed Central 2016-10-06 /pmc/articles/PMC5053148/ /pubmed/27716071 http://dx.doi.org/10.1186/s12876-016-0541-1 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Yamakawa, Kohei
Yoshino, Takuya
Watanabe, Kotaro
Kawano, Koichiro
Kurita, Akira
Matsuzaki, Naomi
Yuba, Yoshiaki
Yazumi, Shujiro
Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title_full Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title_fullStr Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title_full_unstemmed Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title_short Effectiveness of cyclosporine as a treatment for steroid-resistant Cronkhite-Canada syndrome; two case reports
title_sort effectiveness of cyclosporine as a treatment for steroid-resistant cronkhite-canada syndrome; two case reports
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5053148/
https://www.ncbi.nlm.nih.gov/pubmed/27716071
http://dx.doi.org/10.1186/s12876-016-0541-1
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