Histologic Features Can Distinguish Non-Celiac Causes of Villous Atrophy from Those of Celiac Disease
Salwan J Almashat, Kumar Pallav, Daniel Leffler, Ciaran P Kelly, Jeffrey D Goldsmith, Robert M Najarian. Beth Israel Deaconess Medical Center & Harvard Medical School, Boston, MA; Beth Israel Deaconess Medical Center, Boston, MA; University of Mississippi Medical Center, Jackson, MS
Background: Villous atrophy and crypt hyperplasia are relatively specific histologic features of celiac disease (CD). These features are additionally found in patients with other diseases and result in misdiagnoses and inappropriate treatment recommendations. No studies to date have systematically examined the histologic features that can aid the pathologist in recognizing non-celiac enteropathies (NCE) and how they differ from celiac disease.
Design: 32 patients (mean 56 y; 69% F), with negative genetic and serologic tests for CD, but with duodenal biopsies demonstrating villous atrophy were identified by clinical referral. 58 biopsies from these patients were evaluated for the following: mean number of intraepithelial lymphocytes (IEL); presence of intraepithelial lymphocytosis (defined as >30 lymphocytes/100 enterocytes); active neutrophilic inflammation (AI); villus/crypt ratio (VCR); and other histologic features. Indications for endoscopy, comorbidities, serologic data, related extraintestinal biopsy findings, and final clinical diagnoses were obtained following the blinded biopsy review. 16 patients (mean 45 y; 69% F) with clinical and histologic evidence of CD were used as a control group for comparison.
Results: The proportion of NCE biopsies with AI was twice that of CD biopsies (62 vs 31%; p=0.0173), with CD patients never demonstrating involvement of deep crypts. In contrast, CD biopsies had a higher number of mean IEL when compared to NCE (66 vs 33/100 enterocytes; p<0.0001), with only half of NCE biopsies having intraepithelial lymphocytosis. CD biopsies also had lower VCR than those of NCE (0.3 vs. 0.7; p=0.008). Foveolar metaplasia and lymphoid aggregates were seen in both groups without significant differences. Over a mean follow-up period of 30 months, 14 NCE patients were found to have an immune-related disorder, including post-viral and de novo autoimmune enteropathy, as potential causes of their villous atrophy. Additionally, peptic duodenitis, collagenous sprue, CVID, Crohn's, bacterial overgrowth, eosinophilic gastroenteritis, and food allergy were found in 6, 4, 3, 2, 1, 1, and 1 patients, respectively.
Conclusions: The presence of AI, especially that found within crypts, and the absence of intraepithelial lymphocytosis are features that suggest causes of duodenal villous atrophy other than celiac disease.
Monday, March 4, 2013 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 89, Monday Morning