Primary sclerosing cholangitis and celiac disease. A novel association

J. E. Hay, R. H. Wiesner, R. G. Shorter, Nicholas F La Russo, W. P. Baldus

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Abstract

The association of primary sclerosing cholangitis and celiac disease was observed in three patients, an association not previously reported. All three patients were men who presented with chronic cholestatic liver disease at ages 32, 46, and 62 years, respectively. In each patient, endoscopic retrograde cholangiography showed the typical findings of primary sclerosing cholangitis. Histologic features of liver biopsy were compatible with the diagnosis. Two patients had associated chronic ulcerative colitis. All three patients complained of frequent loose stools and weight loss; subsequent testing showed severe steatorrhea (204 to 323 mmol/d of fecal fat on a 100 g fat diet). Total villous atrophy was found in all three patients on histologic examination of the small bowel. Celiac disease was diagnosed at the time of presentation in two patients who had primary sclerosing cholangitis and was diagnosed three years after the onset of primary sclerosing cholangitis in the third patient. The celiac disease responded to a gluten-free diet in each patient whereas the primary sclerosing cholangitis was not affected by dietary treatment. The possibility of a chance association of primary sclerosing cholangitis and celiac disease cannot be accurately assessed but seems unlikely given the rarity of both diseases. The relationship between the two diseases remains unknown, although an immunologic connection is suspected. Celiac disease should be considered in the differential diagnosis of severe steatorrhea in patients with primary sclerosing cholangitis.

Original languageEnglish (US)
Pages (from-to)713-717
Number of pages5
JournalAnnals of Internal Medicine
Volume109
Issue number9
StatePublished - 1988

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Sclerosing Cholangitis
Celiac Disease
Steatorrhea
Fats
Gluten-Free Diet
Cholangiography
Ulcerative Colitis
Atrophy
Liver Diseases
Weight Loss
Differential Diagnosis
Diet
Biopsy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Hay, J. E., Wiesner, R. H., Shorter, R. G., La Russo, N. F., & Baldus, W. P. (1988). Primary sclerosing cholangitis and celiac disease. A novel association. Annals of Internal Medicine, 109(9), 713-717.

Primary sclerosing cholangitis and celiac disease. A novel association. / Hay, J. E.; Wiesner, R. H.; Shorter, R. G.; La Russo, Nicholas F; Baldus, W. P.

In: Annals of Internal Medicine, Vol. 109, No. 9, 1988, p. 713-717.

Research output: Contribution to journalArticle

Hay, JE, Wiesner, RH, Shorter, RG, La Russo, NF & Baldus, WP 1988, 'Primary sclerosing cholangitis and celiac disease. A novel association', Annals of Internal Medicine, vol. 109, no. 9, pp. 713-717.
Hay, J. E. ; Wiesner, R. H. ; Shorter, R. G. ; La Russo, Nicholas F ; Baldus, W. P. / Primary sclerosing cholangitis and celiac disease. A novel association. In: Annals of Internal Medicine. 1988 ; Vol. 109, No. 9. pp. 713-717.
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