Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Sep;112(3):186-196.
doi: 10.32074/1591-951X-157.

Celiac disease: histology-differential diagnosis-complications. A practical approach

Vincenzo Villanacci  1 , Alessandro Vanoli  2   3 , Giuseppe Leoncini  4 , Giovanni Arpa  2   3 , Tiziana Salviato  5 , Luca Reggiani Bonetti  5 , Carla Baronchelli  1 , Luca Saragoni  6 , Paola Parente  7
Affiliations
Review

Celiac disease: histology-differential diagnosis-complications. A practical approach

Vincenzo Villanacci et al. Pathologica. 2020 Sep.

Abstract

Celiac disease is a multi-factorial chronic inflammatory intestinal disease, characterized by malabsorption resulting from mucosal injury after ingestion of wheat gluten or related rye and barley proteins. Inappropriate T-cell-mediated immune response against ingested gluten in genetically predisposed people, leads to characteristic histological lesions, as villous atrophy and intraepithelial lymphocytosis. Nevertheless, celiac disease is a comprehensive diagnosis with clinical, serological and genetic characteristics integrated with histological features. Biopsy of duodenal mucosa remains the gold standard in the diagnosis of celiac disease with the recognition of the spectrum of histological changes and classification of mucosa damage based on updated Corazza-Villanacci system. Appropriate differential diagnosis evaluation and clinical context also for the diagnosis of complications is, moreover, needed for correct histological features interpretation and clinical management.

Keywords: celiac disease; gluten; small bowel; sprue.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest

The Authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
(A-B): normal duodenal mucosa; villous/crypt ratio over 3:1; number of T lymphocytes < 25 x 100 epithelial cells. (A) H&E x 10, (B) CD3 immunostain x 10. (C-D): Type 1 - Grade A lesion; normal villi but with pathological increase of T lymhocytes > 25 x 100 epithelail cells. (C) H&E x 20, (D) CD3 immunostain x 20. (E-F): mild to moderate villous atrophy Type 3A-3B - Grade B1 with pathological increase of T lymphocytes. (E) H&E x 20, (F) CD3 immunostain x 20. (G-H): severe villous atrophy Type 3C - Grade B2 with pathological increase of T lymphocytes. (G) H&E x 20, (H) CD3 immunostain x 20.
Figure 2.
Figure 2.
(A-B): normal villi; T lymphocytes < 25 x 100 epithelial cells. (A) H&E x 10, (B) CD3 immunostain x 10. (C-D): cluster of T Lymphocytes in the superficial epithelium. (C-D) CD3 immunostain x 60 red rectangle. (E-F): linear disposition of T lymphocytes in the deeper part of the mucosa. CD3 immunostain x 4 red rectangle. (G-H): eosinophils in lamina propria.
Figure 3.
Figure 3.
(A-B): collagenous sprue; pathological increase in the thickness of the connective tissue band under the superficial epithelium > 10 mμ; (A) H&E x 20; (B) Trichrome stain x 20. (C-D): refractory celiac disease; pathological increase of T lymphocytes CD3 positive (C) negativity for CD8 (D). C-D x 20. (E-F): enteropathy type T cell lymphoma; (E) x 4, (F) H&E x 40; (G) CD3 immunostain x 40.
See this image and copyright information in PMC

References

    1. Robert ME, Crowe SE, Burgart L, et al. . Statement on best practices in the use of pathology as a diagnostic tool for celiac disease: a guide for clinicians and pathologists. Am J Surg Pathol 2018;42:e44-e58. https://doi.org/10.1097/PAS.0000000000001107 10.1097/PAS.0000000000001107 - DOI - PubMed
    1. Volta U, Caio G, Stanghellini V, et al. . Non-celiac gluten sensitivity: questions still to be answered despite increased awarness. Cell Mol Immunol 2013;10:383-92. https://doi.org/10.1038/cmi.2013.28 10.1038/cmi.2013.28 - DOI - PMC - PubMed
    1. Caio G, Volta U, Sapone A, et al. . Celiac disease: a comprehensive current review. BMC Medicine 2019;17:142-62. https://doi.org/10.1186/s12916-019-1380-z 10.1186/s12916-019-1380-z - DOI - PMC - PubMed
    1. Ludvigsson JF, Rubio-Tapia A, van Dyke CT, et al. . Increasing incidence of celiac disease in a North American population. Am J Gastroenterol 2013:108:818-24. https://doi.org/10.1038/ajg.2013.60 10.1038/ajg.2013.60 - DOI - PMC - PubMed
    1. Mustalahti K, Catassi C, Reunanen A, et al. . The prevalence of celiac disease in Europe: results of a centralized, international mass screening project. Ann Med 2010;42:587-95. https://doi.org/10.3109/07853890.2010.505931 10.3109/07853890.2010.505931 - DOI - PubMed