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Neutrophilic Infiltration in Gluten-sensitive Enteropathy Is Neither Uncommon Nor Insignificant: Assessment of Duodenal Biopsies From 267 Pediatric and Adult Patients

Moran, Christopher J. MD*,†; Kolman, Olga K. MD; Russell, Gary J. MD*,†; Brown, Ian S. MD§; Mino-Kenudson, Mari MD‡,#

American Journal of Surgical Pathology: September 2012 - Volume 36 - Issue 9 - p 1339–1345
doi: 10.1097/PAS.0b013e318254f413
Original Articles

The histologic findings of celiac disease, that is, gluten-sensitive enteropathy (GSE), are dominated by increased intraepithelial lymphocytes, villous blunting, lymphoplasmacytic infiltration of lamina propria, and crypt hyperplasia. To date, neutrophils have not been thought to constitute a significant cell type in GSE, and their presence often invokes consideration of alternative diagnoses. Thus, we sought to determine the prevalence and severity of neutrophilic infiltration in duodenal biopsies from patients with GSE. The degree of neutrophilic infiltration and features characteristic of GSE were assessed in duodenal biopsies from 267 clinically confirmed GSE patients (116 adults and 151 children). These specimens were graded by the disease activity score (DAS) and the neutrophilic activity score (NAS). Gastric antral biopsies obtained from 195 patients were also evaluated for lymphocytic gastritis. NAS was correlated with DAS and other clinicopathologic features. We found that 56% of pediatric and 28% of adult GSE patients had significant duodenal neutrophilia. NAS was higher in children than in adults (2.3 vs. 1.2, P<0.001). Multivariate regression showed that DAS, eosinophilic infiltration, and foveolar metaplasia correlated positively, and age correlated negatively with NAS. Lymphocytic gastritis was seen in 21.5% of the gastric biopsies. The presence of lymphocytic gastritis correlated positively with NAS and DAS, and in the pediatric population it correlated negatively with age. Significant duodenal neutrophilia is often found in patients with celiac disease, especially in the pediatric population, and is associated with more active disease. Thus, the findings of duodenal neutrophilia in biopsies, otherwise consistent with GSE, should not preclude the diagnosis of GSE.

*Department of Pediatrics, Mass General Hospital for Children

Department of Pathology, Massachusetts General Hospital

Department of Pediatrics, Harvard Medical School

#Department of Pathology, Harvard Medical School, Boston, MA

§Sullivan Nicolaides Pathology, Taringa, Qld, Australia

C.J.M. and O.K.K., and I.S.B. and M.M-K. contributed equally.

Presented in part at the 99th annual meeting of the United States and Canadian Academy of Pathology in Washington, DC.

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Mari Mino-Kenudson, MD, Department of Pathology, Massachusetts General Hospital, 55 Fruit Street, Warren 122, Boston, MA 02114 (e-mail: mminokenudson@partners.org).

© 2012 Lippincott Williams & Wilkins, Inc.