ACR Codes: 7.9
Celiac disease, also know as celiac sprue, adult celiac disease, nontropical sprue, and gluten-sensitive enteropathy, is characterized by damage to the mucosa of the small intestine and malabsorption of most nutrients. The disease is activated by the dietary ingestion of wheat gluten and similar proteins in rye, barley, and oats.(1) Celiac disease is predominantly a disease found in Europe and in those places to which Europeans have migrated. Recent European studies with population screening by serological tests, followed where appropriate by intestinal biopsy, reported adult prevalence rates of 1 in 150 in Ireland[2] and 1 in 300 in Finland.[3] Celiac disease has traditionally been regarded as an uncommon disease in North America but screening tests suggest that celiac disease may be underdiagnosed in the United States.[4] A study in Maryland found antiendomysium antibodies, a specific test for celiac disease, in 1 in 250 blood donors.[5]
The clinical presentation of celiac disease can vary markedly, particularly in adults. At one end of the spectrum, patients may present with diarrhea characterized by large, bulky, pale, foul-smelling stools that have a loose consistency and float because of an increased content of air and fat. Abdominal pain is unusual and, in patients with long-standing celiac disease, should invoke the consideration of a complicating lymphoma. At the other end of the spectrum, adults may be relatively asymptomatic and present with only nonspecific complaints such as malaise, lassitude, and weekness. Children often present with failure to gain weight, diarrhea, and abdominal distention. (1)
Nonspecific radiologic findings in celiac disease include:
1. Moulage sign: a tendency for barium to clump into smooth, elongated masses in the proximal jejunum; clumps represent flocculations of previously used barium suspensions
2. lumen dilatation defined as more than 30 mm
3. luminal fluid excess which may be secondary to any hypersecretory state
4. fold thickening which occurs only when there is associated edema, as in hypoalbuminemia
5. painless, transient intussusceptions
Specific Radiologic Findings
1. Jejunal Folds: increased separation of the mucosal folds; five folds or more per incch is a normal finding and renders a diagnosis of celiac disease unlikely
2. Ileal Folds: A reversal of the normal fold character between ileum and jejunum; there are fewer jejunal folds per unit length with an increase in the ileal folds from two to four per inch to four to six per inch.
3. Mosaic Pattern: Network of barium-containing grooves separated areas 1 to 3 mm in diameter
4. Duodenal Folds: Folds can be fewer in number and appear irregular, especially in the distal duodenum.(6)
The gold standard for the diagnosis of celiac disease consists of the characteristic, although not specific, changes shown by histologic study of biopsy specimens of duodenal or jejunal mucosa. Their causal relationship to celiac disease must be confirmed by a favorable clinical response to a gluten-free diet and, preferably also, by reversion to near normality on a follow-up mucosal biopsy. (6)
Celiac disease appears to be identical in adults and children. Adult celiac disease maybe an extension of the childhood disease, separated by years of latency, or it may be a new disease. (6)
Reference(s): 1. Yamada, T., Alpers, D, Powell, D., et al. Textbook of Gastroenterology Second Edition. J.B. Lippincott Company. Copyright 1995. pp. 1643-1648.
2. Johnston SD, Watson RGP, McMillan SA et al. Preliminary results from follow-up of a large scale population survey of antibodies to gliadin, reticulin and endomysium. Acta Paediatr Suppl 1996;412:61-64.
3. Collin P, Reunala T, Rasmussen M et al. High incidence and prevalence of adult celiac disease. Augmented diagnostic approach. Scand J Gastroenterol 1997 32:1129-33.
4. Ferguson A. Celiac disease: An eminently treatable condition, may be underdiagnosed in the United States. Am J Gastroenterol 1997;92:1252-1254.
5. Not T, Horvath K, Hill ID et al. Endomysium antibodies in blood donors predicts a high prevalence of celiac disease in the USA. Gastroenterology 1996;110:A351.
6. Gore, R., Levine, M., Lauger, I. Textbook of Gastrointestinal Radiology. W.B. Saunders Company. Copyright 1994. pp.
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