Nomenclature and diagnosis of seronegative celiac disease and chronic non-celiac enteropathies in adults

Abstract

Small bowel villous atrophy is one of the main histopathological manifestations of chronic enteropathy. In most cases, this condition is caused by celiac disease. Celiac disease is an autoimmune disease that occurs in genetically predisposed individuals and is characterized by damage to the mucous membrane of the small intestine (T-cell-mediated enteropathy) due to the influence of gluten and is treated with the help of a lifelong gluten-free diet. Diagnosis of classic celiac disease does not cause difficulties. Diagnostic algorithms have been developed for years. Diagnostic problems arise when small bowel villous atrophy occurs in patients who have gastrointestinal symptoms but are negative for celiac disease-specific antibodies. Differential diagnosis of small bowel villous atrophy without serological markers for celiac disease in adults includes seronegative celiac disease and chronic non-celiac enteropathies. There has long been diagnostic and clinical uncertainty about these conditions. This uncertainty has led to misdiagnoses and increased the number of people following a gluten-free diet unnecessarily. Between September 2019 and July 2021, an international working group of 13 gastroenterologists from six countries worked to create a consensus on the nomenclature and diagnosis of seronegative celiac disease and non-celiac enteropathies in adults.