Melissa P Upton1. 1. Department of Pathology, University of Washington Medical Center, Seattle, WA 98195-6100, USA. mupton@u.washington.edu
Abstract
CONTEXT: Celiac sprue affects genetically susceptible individuals, who develop small intestinal injury and malabsorption following dietary exposure to gluten. The histologic features are nonspecific but characteristic. OBJECTIVES: To outline the histologic features of celiac sprue and the necessary clinical context to permit a diagnosis of celiac sprue, to assist the pathologist to identify artifactual biopsy changes that may mimic sprue, to define the differential diagnosis for conditions with a similar histology, and to review historic investigations of this disease. DATA SOURCES: Sources include the historic experiments and clinical work of members of the Gastroenterology Division of the Department of Medicine and experiences with gastrointestinal pathology consultation material at the University of Washington, Seattle, with reference to selected peer-reviewed articles. CONCLUSIONS: Confirmation of a diagnosis of celiac sprue is 2-fold: first, biopsy evidence of a characteristic, but nonspecific, pattern of injury including villous blunting or flattening, surface enterocyte damage, and increased intraepithelial lymphocytes; and second, dramatic clinical response to a gluten-free diet. Complete gluten removal from the diet is effective treatment for patients with symptoms of malabsorption; however, lifelong adherence to the diet is expensive, socially limiting, and nearly impossible on a contemporary diet with manufactured foodstuffs. Therefore, pathologists should avoid overdiagnosis of celiac disease based on minimal, nonspecific histologic changes.
CONTEXT: Celiac sprue affects genetically susceptible individuals, who develop small intestinal injury and malabsorption following dietary exposure to gluten. The histologic features are nonspecific but characteristic. OBJECTIVES: To outline the histologic features of celiac sprue and the necessary clinical context to permit a diagnosis of celiac sprue, to assist the pathologist to identify artifactual biopsy changes that may mimic sprue, to define the differential diagnosis for conditions with a similar histology, and to review historic investigations of this disease. DATA SOURCES: Sources include the historic experiments and clinical work of members of the Gastroenterology Division of the Department of Medicine and experiences with gastrointestinal pathology consultation material at the University of Washington, Seattle, with reference to selected peer-reviewed articles. CONCLUSIONS: Confirmation of a diagnosis of celiac sprue is 2-fold: first, biopsy evidence of a characteristic, but nonspecific, pattern of injury including villous blunting or flattening, surface enterocyte damage, and increased intraepithelial lymphocytes; and second, dramatic clinical response to a gluten-free diet. Complete gluten removal from the diet is effective treatment for patients with symptoms of malabsorption; however, lifelong adherence to the diet is expensive, socially limiting, and nearly impossible on a contemporary diet with manufactured foodstuffs. Therefore, pathologists should avoid overdiagnosis of celiac disease based on minimal, nonspecific histologic changes.
Authors: Giacomo Caio; Umberto Volta; Anna Sapone; Daniel A Leffler; Roberto De Giorgio; Carlo Catassi; Alessio Fasano Journal: BMC Med Date: 2019-07-23 Impact factor: 8.775