Medical Management of Cronkhite-Canada Syndrome

Eric Ward, MD, Herbert C. Wolfsen, MD, Christine Ng, MD

Disclosures

South Med J. 2002;95(2) 

In This Article

Discussion

Cronkhite-Canada syndrome usually follows a progressive course with a high rate of mortality due to complications such as anemia, gastrointestinal bleeding, congestive heart failure, and septicemia.[3] The optimal therapy for this disease is not known, in part because the disease is so rare. However, our patient has had a sustained symptomatic response to the novel combination therapy of a corticosteroid, H1- and H2-receptor blockers, and cromolyn sodium. Corticosteroids, ranitidine, and antibiotics have been used individually for the treatment of CCS, but the use of this combination of medications has not previously been reported in the medical literature. This regimen was based on the presence of mast cells seen on histopathologic analysis of intestinal biopsies and the possible role of mast cell dysfunction in the pathogenesis of CCS. Stabilization of the mast cells by cromolyn sodium and the antihistamine effects of loratadine and ranitidine may account for this regimen's effectiveness if indeed mast cell dysfunction is involved in the development of the syndrome. However, without the concomitant use of corticosteroids, these medications have been unable to maintain clinical remission. Our patient's response to this combination therapy may provide insight into the optimal treatment for this progressive and usually fatal disease.

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