Successful treatment of Cronkhite–Canada syndrome using anti-tumor necrosis factor antibody therapy

Watanabe, Daisuke; Ooi, Makoto; Hoshi, Namiko; Kohashi, Michitaka; Yoshie, Tomoo; Ikehara, Nobunao; Yoshida, Masaru; Yanagita, Emmy; Yamasaki, Takashi; Itoh, Tomoo; Azuma, Takeshi

doi:10.1055/s-0034-1377539

Cited by 20 publications

(16 citation statements)

References 3 publications

(2 reference statements)

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“…Current CCS treatments used individually or in combination include steroids [1, 11, 17, 18, 24], nutritional therapy [25], 5-aminosalicylate acid [26], histamine H 2 receptor antagonists [27], anti-tumor necrosis factor α (TNF-α) agents [28], immunomodulators [28], and eradication of Helicobacter pylori [29]. Steroids are considered the mainstay of medical treatment, although the recommended dose and duration of their use have varied widely in the literature, with no current “gold standard” [11, 17, 18, 30].…”

Section: Introductionmentioning

confidence: 99%

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey

et al. 2015

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BackgroundFirst 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.MethodsA 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.ResultsThe 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.ConclusionsThe 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.Electronic supplementary materialThe online version of this article (doi:10.1007/s00535-015-1107-7) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey

et al. 2015

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“…In contrast, including our case, there are now three reports of successful treatment of CCS as an inflammatory process with a dramatic response to a tumor necrosis factor (TNF) inhibitor(3, 4). The high disease mortality associated with CCS and early success reported with TNF inhibitors in these cases demand that this approach should be investigated as a first-line therapy(5).…”

Section: Dear Editormentioning

confidence: 76%

Cronkhite Canada Syndrome: Significant Response to Infliximab and a Possible Clue to Pathogenesis

Boland

Bagi

Valasek

et al. 2016

American Journal of Gastroenterology

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“…To improve the poor prognosis of CCS, various treatments have been proposed, such as nutritional support, antibiotics, corticosteroids, immunomodulators, biologics, and surgery [4]. Corticosteroids are reported to be highly effective for treating CCS [2, 5].…”

Section: Discussionmentioning

confidence: 99%

“…On the other hand, the remaining 9 patients (16 %) did not respond clinically to corticosteroids. (Table 1) [2, 4, 7–11]. Among the 9 steroid-resistant cases, 7 cases described the clinical course after the treatment with corticosteroids and 2 cases didn’t.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2016

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BackgroundCronkhite-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 presentationOur 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.ConclusionsTreatment with calcineurin inhibitor, such as CyA, could be a potential option for steroid-resistant CCS.Electronic supplementary materialThe 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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Successful treatment of Cronkhite–Canada syndrome using anti-tumor necrosis factor antibody therapy

Cited by 20 publications

References 3 publications

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey

Endoscopic and clinical evaluation of treatment and prognosis of Cronkhite–Canada syndrome: a Japanese nationwide survey

Cronkhite Canada Syndrome: Significant Response to Infliximab and a Possible Clue to Pathogenesis

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

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