Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey
Open Access
- 28 July 2015
- journal article
- research article
- Published by Springer Science and Business Media LLC in The Esophagus
- Vol. 51 (4), 327-336
- https://doi.org/10.1007/s00535-015-1107-7
Abstract
Background First reported in 1955, Cronkhite–Canada syndrome (CCS), a rare syndrome characterized by ectodermal abnormalities and inflammatory changes of the gastrointestinal tract mucosa, has been associated with a poor prognosis and life-threatening malignant complications. In a large population survey, we endeavored to characterize the course and treatment outcome of CCS through clinical and endoscopic assessment, and to explore its optimal treatment and surveillance strategy. Methods A retrospective analysis of 210 patients with CCS was conducted via a questionnaire-based nationwide survey of 983 teaching hospitals located throughout Japan. We assessed clinical features, endoscopic findings, treatments used, and short- and long-term outcomes. Results The average age at diagnosis was 63.5 years. In all cases, upper or lower gastrointestinal tract polyposis was confirmed, accompanied by characteristic ectodermal abnormalities. Of the treatments used, oral corticosteroids (30–49 mg/day) were the most effective treatment for active disease, with adjunctive nutritional support considered beneficial. With corticosteroid treatment, abdominal symptoms were relieved within a few months, whereas polyp regression often required more than 6 months. Maintenance of endoscopic remission with or without steroids for 3 years significantly lowered the development of CCS-related cancer, compared with relapsers or nonresponders, underscoring the importance of sustained endoscopic remission for cancer prevention. Conclusions The prognosis of CCS has greatly improved through the use of improved medical treatment. Although CCS continues to be relentlessly progressive, carrying a high cancer risk, a sufficient dose and duration of corticosteroid therapy accompanied by nutritional support and periodic endoscopic surveillance appears to improve its natural history.Keywords
Funding Information
- Grant-in -aid for the Intractable Disease Project of the Ministry of Health, Labour, and Welfare of Japan
- Grants-in-Aid for Scientific Research from the Japanese Ministry of Education, Culture, Sports, Science and Technology (26460980)
This publication has 47 references indexed in Scilit:
- Histopathological insights into hair loss in Cronkhite–Canada syndrome: Diffuse anagen‐telogen conversion precedes clinical hair loss progressionAustralasian Journal of Dermatology, 2013
- Clinicopathologic Features and Treatment Outcomes in Cronkhite–Canada Syndrome: Support for AutoimmunityDigestive Diseases and Sciences, 2011
- A fulminant course of Cronkhite-Canada syndromeEndoscopy, 2010
- A case of Cronkhite–Canada syndrome presenting with adenomatous and inflammatory colon polypsNature Reviews Gastroenterology & Hepatology, 2010
- Hamartomatous Polyposis SyndromesSurgical Clinics of North America, 2008
- A Case of Cronkhite–Canada Syndrome markedly improved with mesalazine therapyDigestive Endoscopy, 2003
- Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritisThe Esophagus, 2003
- The Cronkhite-Canada Syndrome with Focus on Immunity and Infection Report of a CaseJournal of Clinical Gastroenterology, 1987
- Cronkhite Canada syndrome: a new hypothesis.Gut, 1985
- The Cronkhite-Canada syndrome: an ultrastructural study of pathogenesis.Journal of Clinical Pathology, 1985