Gluten-sensitive enteropathy (celiac disease): controversies in diagnosis and classification

Arch Pathol Lab Med. 2010 Jun;134(6):826-36. doi: 10.5858/134.6.826.


Context: Celiac disease, or gluten-sensitive enteropathy, is a chronic inflammatory disorder of the small intestine characterized by malabsorption after ingestion of gluten in individuals with a certain genetic background. Clinical presentation can vary from full-blown malabsorption to subtle and atypical symptoms. Diagnosis currently relies on clinicopathologic studies including mucosal biopsy, serologic tests, and the effects of a diet free of gluten on the symptoms. Mucosal pathologic features are also variable, ranging from mild abnormalities, including intraepithelial lymphocytosis, to completely flat mucosa. Since patients with minimal histologic lesion of intraepithelial lymphocytosis often present with normal serologic findings, biopsy diagnosis becomes more important for identifying such individuals. Classification of mucosal pathology in gluten-sensitive enteropathy has been a subject of controversy among pathologists and needs to be revised according to the current understanding of the disease.

Objectives: To highlight the variations in clinical and pathologic presentation of gluten-sensitive enteropathy, to emphasize the importance of small-intestinal biopsy evaluation in the diagnosis, and to propose a new classification of mucosal pathology in gluten-sensitive enteropathy, in an effort to overcome the problems related to the classification systems currently available.

Data sources: A review of the literature on clinicopathologic features and the morphologic spectrum of gluten-sensitive enteropathy is presented.

Conclusions: Considering that there are many entities in the differential diagnosis of gluten-sensitive enteropathy, because of the varied clinicopathologic spectrum of the disease, diagnosis depends on good clinicopathologic communication. The classification that is presented in this review is a simple and practical approach to improve clinicopathologic correlation in gluten-sensitive enteropathy.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Celiac Disease / classification*
  • Celiac Disease / diagnosis*
  • Celiac Disease / pathology
  • Diagnosis, Differential
  • Humans
  • Intestinal Mucosa / pathology
  • Lymphocytosis / pathology