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Patchy Duodenal Atrophy or Proximal Duodenal Involvement by Celiac Disease?

Drut, Ricardo; Rúa, Eduardo Cueto

Journal of Pediatric Gastroenterology and Nutrition: August 2004 - Volume 39 - Issue 2 - p 216-217
Letters to the Editor
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Hospital de Niños, La Plata, Argentina

To the Editor: We read with interest the article by Bonamico et al. (1) describing their finding of “patchy” villous atrophy of the duodenum in childhood celiac disease. What the authors describe as patchy villous atrophy appears to be atrophic changes of the mucosa of the duodenal mucosa mainly in the bulb. This nonuniform involvement of the mucosa of the duodenum with normal mucosa and abnormal areas in juxtaposition has been described previously in celiac disease. The use of the term “patchy” atrophy seems only to increase the general confusion about the meaning of this histologic finding. The article raises another question. How do the authors explain the symptoms of their patients if only the duodenal bulb is affected by the mucosal atrophy? They state in the article, “It is worth noting that the clinical presentation did not seem to relate to the histologic findings of patchiness. In fact, most of our patients presented with typical symptoms.”

The hypothesis of patchy atrophy has been used to explain the presence of autoantibodies in patients with normal small bowel biopsy specimens. With the endoscope we do occasionally see areas of small bowel with a cobblestone appearance next to areas that appear grossly normal. More frequently, we see different degrees of atrophy in different areas of the duodenum, a fact that is stressed in the article by Bonamico et al. (1). This, in our opinion, should not be named patchy atrophy.

This article may prove useful to better define the terminology used when dealing with celiac disease. Perhaps it is worthwhile to redefine patchy atrophy. We suggest that such a descriptive term should be restricted to true patches of atrophy recognized endoscopically and histologically within the duodenum or proximal jejunum. This finding, as far as we are aware, has not been described in celiac disease. Proper orientation and interpretation of endoscopically obtained material has been stressed in the literature (2). In our opinion, the best biopsies are taken with the conventional Crosby capsule. The author’s statement (1) “that, although almost half of endoscopic samples presented some difficulties with histologic analysis, the sample was felt to be adequate in 98% of cases to make correct diagnosis” indicates the subjectivity in interpretation that is the rule in interpretation of mucosal biopsy specimens.

We find the Marsh classification used in this article to be subjective. Our experience with more than 8,000 small bowel biopsy specimens is described in a previously published report (3), and our grading system is summarized below.

  • Grading of villous atrophy by villous/crypt (V/C) ratio
  • Normal mucosa: V/C ratio, >2.5
  • Grade 1 atrophy: V/C ratio, 2.5–2
  • Grade 2 atrophy: V/C ratio, 1–2
  • Grade 3 atrophy: V/C ratio, 1–0.5
  • Grade 4 atrophy: V/C ratio, <0.5

We recommend that crypt and villous length be measured in an area of the biopsy in which at least two to three crypts are completely oriented from top to bottom. Our experience is that atrophy of grades 3 or 4 is characteristic of untreated celiac disease. Grade 2 villous atrophy is usually seen in patients with poor adherence to gluten-free diet. Strict adherence to a gluten-free diet usually results in villous atrophy grade 1 or normal V/C ratio.

Ricardo Drut

Eduardo Cueto Rúa

Hospital de Niños, La Plata, Argentina

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REFERENCES

1. Bonamico M, Mariani P, Thanasi E, et al. Patchy villous atrophy of the duodenum in childhood celiac disease. J Pediatr Gastroenterol Nutr 2004;38:204–7.
2. Shidrawi RG, Przemiosio P, Davies DR, et al. Pitfalls in diagnosing coeliac disease. J Clin Pathol 1994;47:693–4.
3. Drut R, Cueto Rúa E. The histopathology of celiac disease: order must prevail out of chaos. Int J Surg Pathol 2001;9:261–4.
© 2004 Lippincott Williams & Wilkins, Inc.