Abstract
Although villous atrophy is the hallmark of coeliac disease and its complications, this lesion can manifest in other etiologically heterogeneous enteropathies unrelated to gluten ingestion. Consequently, the differential diagnosis and clinical management of seronegative villous atrophy are challenging. Seronegative coeliac disease is the most frequent cause of serology negative villous atrophy, but it is still a poorly defined clinical entity, whose diagnosis relies on the clinical and histological response to a gluten-free diet after the exclusion of other causes of villous atrophy. Correct identification and targeted management of these enteropathies with seronegative villous atrophy is mandatory to contrast the potentially significant effects on long-term morbidity and mortality. In this chapter, we aim to summarize the current evidence for the investigation and clinical management of seronegative villous atrophy.
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