Premises: Autoimmune enteropathy (AIE) is a rare cause of villous atrophy, rarely seen in adults.Case description: A 71-year-old patient with hypertension on olmesartan was admitted for chronic non-bloody diarrhea, abdominal pain and weight loss of 15 kg. Serology for celiac disease was negative. ARBs were immediately discontinued on suspicion of olmesartan-related enteropathy. However, rapid worsening of conditions with MOF due to malnutrition, persistence of diarrhea and acute kidney failure, required ICU monitoring. Antibiotic therapy was ineffective leading to high-doses of methylprednisolone approach due to severity of illness. EGD and colonoscopy showed severe atrophy in ileum and duodenum. Histology was not pathognomonic but suspicious for Crohn’s or celiac disease although a rare form enteropathy was considered. Meanwhile, common immunodeficiency, refractory celiac disease (no specific HLA alleles), T-lymphoma associated enteropathy, CMV were excluded. Search for anti-enterocyte antibodies was negative. Hospitalization was long and rich of complications (e.g. candidemia) but histological remission of atrophy after steroids and clinical improvement were seen so diagnosis of autoimmune enteropathy was made.Conclusions: Criteria for AIE diagnosis include histological findings and absence of other causes of villous atrophy. Gut-specific antibodies are no longer required for diagnosis. Medications (i.e. olmesartan, NSAID) can trigger secondary form. Diagnosis remains challenging, but it should be considered in villous atrophy without positive celiac serology.
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