Successful Treatment of Refractory Sprue with Cyclosporine

  1. George F. Longstreth, MD
  1. From Southern California Permanente Medical Group, San Diego, California. Requests for Reprints: George F. Longstreth, MD, Kaiser Medical Center, 4647 Zion Avenue, San Diego, California 92120. Acknowledgment: The author thanks Dr. William Cory for substantial contributions to the patient's care, Dr. Naomi Buckwalter for pathology consultation and the photographs, Dr. Martin F. Kaghoff for DNA typing.

    Celiac sprue is a disorder of absorption that is identified by characteristic histologic abnormalities in the small-bowel mucosa [1, 2]. The clinical and histologic features respond to a gluten-free diet. Patients with refractory sprue do not respond to strict gluten avoidance and have a poor prognosis [3]. A patient with severe sprue who was unresponsive to a gluten-free diet and corticosteroid therapy experienced remission after receiving cyclosporine therapy.

    Case Report

    A 45-year-old woman was hospitalized in January 1986 because of diarrhea (5 to 24 stools/d) for 5 months, vomiting for 6 weeks, and a weight loss of 21 kg. She was 1.64 m in height, weighed 95 kg, and had generalized weakness. Results of laboratory tests of blood and urine were unremarkable. Stool examination for pathogens and an alkalinization test for phenolphthalein-containing laxative were negative. The stool volume was 814 to 2014 mL/d. While on a diet containing 100 g of fat, she excreted 9.4 g fat/d. The results of sigmoidoscopic evaluation and a small-bowel roentgenographic series were normal. Esophagogastroduodenoscopic evaluation showed mild duodenal hyperemia, and findings in a duodenal biopsy specimen were typical of sprue (Figure 1, panel A). Treatment included a gluten-free diet and prednisone (initially 60 mg/day).

    Figure 1. Initial biopsy specimen showing total villous atrophy, crypt hyperplasia, flattened surface cells, and an intense inflammatory infiltrate in the lamina propria. Goblet cells are absent. Biopsy specimen obtained after the patient had been on a gluten-free diet for 2.5 years, showing persistence of abnormalities typical of sprue. Biopsy specimen obtained after cyclosporine use for 1 year, showing villi with a villous:crypt ratio of 1:1, columnar surface cells, a normal number of goblet cells, and less inflammation. Biopsy specimen obtained 3 years after cyclosporine therapy was discontinued, showing no abnormality. The villous:crypt ratio is 3:1 (hematoxylin and eosin). Duodenal biopsy findings in a patient with sprue.Panel APanel BPanel CPanel D

    During the next 6 months, she was hospitalized seven times for diarrhea, vomiting, and fluid …

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    1. Ann Intern Med November 15, 1993 vol. 119 no. 10 1014-1016
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