Review ArticlesCeliac Disease: Updates on Pathology and Differential DiagnosisDai, Yinghuan MD, PhD*; Zhang, Qin MD†; Olofson, Andrea M. MD‡; Jhala, Nirag MD§; Liu, Xiuli MD, PhD‡Author Information *Department of Pathology, Second Xiangya Hospital of Central South University, Changsha †Department of Pathology, the Third Central Hospital of Tianjin, Tianjin, China ‡Department of Pathology, Immunology and Laboratory Medicine, University of Florida, Gainesville, FL §Department of Pathology and Laboratory Medicine, Lewis Katz School of Medicine at Temple University, Philadelphia, PA Y.H. and Q.Z. contributed equally. The authors have no funding or conflicts of interest to disclose. All figures can be viewed online in color at www.anatomicpathology.com. Reprints: Xiuli Liu, MD, PhD, Department of Pathology, Laboratory Medicine, and Immunology, University of Florida, P.O. Box 100275, Gainesville, FL (e-mail: firstname.lastname@example.org). Advances In Anatomic Pathology: September 2019 - Volume 26 - Issue 5 - p 292-312 doi: 10.1097/PAP.0000000000000242 Buy Metrics Abstract Celiac disease is a gluten-triggered immune-mediated disorder, characterized by inflammation of the enteric mucosa following lymphocytic infiltration and eventually resulting in villous blunting. There have been many developments in refining diagnostic laboratory tests for celiac disease in the last decade. Biopsy-sparing diagnostic guidelines have been proposed and validated in a few recent prospective studies. However, despite these developments, histologic evaluation of duodenal mucosa remains one of the most essential diagnostic tools as it helps in the diagnosis of celiac disease in individuals who do not fulfill the biopsy-sparing diagnostic criteria and in those not responding to a gluten-free diet. Histologic evaluation also allows for the assessment of mucosal recovery after treatment and in the identification of concurrent intestinal diseases. Therefore, pathologists should be familiar with the histologic spectrum of celiac disease and need to be aware of other disorders with similar symptoms and histopathology that may mimic celiac disease. This review aims to provide pathologists with updates on celiac laboratory testing, biopsy-sparing diagnostic criteria, histopathology, complications, and differential diagnoses of celiac disease. Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.