Abstract
The differential diagnosis and treatment of seronegative enteropathy, also termed seronegative villous atrophy (SNVA), is a clinical challenge. Although seronegative coeliac disease (CD) is a frequent cause of SNVA, the aetiology can include immune-mediated, inflammatory, infectious, and drug-related forms. As a misdiagnosis of SNVA can result in patients being unnecessarily placed on a lifelong strict gluten-free diet or even given incorrect immunosuppressive therapy, the aim of this paper is to provide an evidence-based and practical approach for the workup and management of SNVA.
Highlights
The Definition of Seronegative EnteropathyAccording to the current definition, certain cases do not meet the criteria for the diagnosis of seronegative villous atrophy (SNVA): for example, the presence of isolated intraepithelial lymphocytosis of the small intestine mucosa (Marsh grade 1) in a seronegative patient [6] or selective IgA deficiency, i.e., where celiac-specific IgA antibodies are absent but specific IgG antibodies (IgG-seropositive coeliac disease) are present
Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, ul
Common variable immune deficiency (CVID)-related enteropathy shares some histological features with coeliac disease (CD): small intestinal villous atrophy, crypt hyperplasia, and intraepithelial lymphocytosis are present in both conditions
Summary
According to the current definition, certain cases do not meet the criteria for the diagnosis of SNVA: for example, the presence of isolated intraepithelial lymphocytosis of the small intestine mucosa (Marsh grade 1) in a seronegative patient [6] or selective IgA deficiency, i.e., where celiac-specific IgA antibodies are absent but specific IgG antibodies (IgG-seropositive coeliac disease) are present. The latter is ruled out in patients receiving a gluten-free diet, those with a significant reduction in the supply of gluten, or those on immunosuppressive treatment [3].
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