title: Autoimmune enteropathy in a 13-year-old celiac girl successfully treated with infliximab.
abstract: Autoimmune enteropathy (AIE) is a rare cause of small bowel villous atrophy, characterized by malabsorption, unresponsiveness to dietary restriction, circulating autoantibodies to enterocytes, and an overall predisposition to autoimmunity. Albeit mainly regarded as a disease of early childhood, several adult-onset AIE cases have been identified. This report describes for the first time the life-threatening clinical presentation and the management of overlapping AIE in a compliant-to-diet young celiac girl. A 13-year-old celiac girl was admitted because of vomiting, weight loss, diarrhea, hypoproteinemia, and neurological disturbances such as head tremors, vertical nystagmus, and lower limb hyperesthesia. Before this, she had always been compliant on a strict gluten-free diet and her medical history was unremarkable. The diagnosis of AIE was established on histologic findings and on the presence of antienterocyte antibodies. She was initially treated with high-dose Methylprednisolone and Azathioprine. However, only Infliximab proved itself as a highly effective tool for achieving clinical remission and restoring small bowel villous architecture.
authors: *Pediatric Gastroenterology and Liver Unit, Department of Pediatrics, University Hospital Umberto I †Department of Clinical Science, Sapienza University of Rome, Rome ‡First Department of Medicine, Fondazione Policlinico S. Matteo, University of Pavia, Pavia, Italy.
authors: Francesco
authors: F
authors: Valitutti
authors: Maria
authors: M
authors: Barbato
authors: Marina
authors: M
authors: Aloi
authors: Adriana
authors: A
authors: Marcheggiano
authors: Giovanni
authors: G
authors: Di Nardo
authors: Stefania
authors: S
authors: Leoni
authors: Donatella
authors: D
authors: Iorfida
authors: Gino R
authors: GR
authors: Corazza
authors: Salvatore
authors: S
authors: Cucchiara
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D001706:Biopsy; D002446:Celiac Disease / Q000175:diagnosis / Q000178:diet therapy* / Q000276:immunology; D055050:Diet, Gluten-Free; D005260:Female; D005765:Gastrointestinal Agents / Q000627:therapeutic use*; D006801:Humans; D000069285:Infliximab; D007583:Jejunum / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D016884:Polyendocrinopathies, Autoimmune / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D000911:Antibodies, Monoclonal; D005765:Gastrointestinal Agents; D000069285:Infliximab