Clinical manifestations and gastrointestinal pathology in 40 patients with autoimmune enteropathy
2019
Abstract
Autoimmune enteropathy(AIE) is a rare condition that may affect pediatric and adult patients, frequently associated with
primary immunodeficiencies. We performed a retrospective study on clinical and histological findings from 40 AIE patients. Histological presentation showed a prevalent celiac disease pattern (50%), followed by the
mixed pattern(35%), independently of age, chronic active duodenitis (10%), and GVHD-like pattern (5%). Patients with
primary immunodeficiencies(24/40) presented mainly with the celiac disease pattern (72.2% versus 22.2%; p
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