title: Mesenchymal stromal cell infusions as rescue therapy for corticosteroid-refractory adult autoimmune enteropathy.
abstract: Adult autoimmune enteropathy (AIE) is a rare cause of malabsorption syndrome unresponsive to dietary restriction. Its diagnostic hallmarks are small-bowel villous atrophy and antienterocyte autoantibodies. Therapy is based mainly on nutritional support and immunosuppression. We treated a 61-year-old woman with corticosteroid-refractory AIE and life-threatening malabsorption syndrome with systemic infusions of autologous, bone marrow-derived, mesenchymal stromal cells (MSCs) as rescue therapy. The MSCs were expanded ex vivo following a previously used Good Manufacturing Practice procedure, and 2 intravenous infusions of 1.8 × 10(6) MSCs/kg body weight were administered 2 weeks apart. Analysis of circulating and mucosal regulatory T-and B-cell numbers, and of serum and secretory immunoglobulin levels, was performed before and after treatment. The MSC infusions were safe and effective, leading to disappearance of disease hallmarks and recovery from the life-threatening condition. Increases in mucosal regulatory T-cell numbers and secretory immunoglobulin levels were also observed. The benefit, however, was transient, and a further MSC infusion resulted in the same short efficacy. This case encourages the use of MSCs to treat patients with life-threatening, corticosteroid-refractory AIE and suggests that MSC infusion can attenuate, albeit transiently, the autoimmune attack.
authors.affiliation: Clinica Medica I, Fondazione Istituto Di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Università degli Studi di Pavia, Italy. rachele.ciccocioppo@unipv.it
authors.forename: Rachele
authors.initials: R
authors.lastname: Ciccocioppo
authors.forename: Maria L
authors.initials: ML
authors.lastname: Russo
authors.forename: Maria E
authors.initials: ME
authors.lastname: Bernardo
authors.forename: Federico
authors.initials: F
authors.lastname: Biagi
authors.forename: Laura
authors.initials: L
authors.lastname: Catenacci
authors.forename: Maria A
authors.initials: MA
authors.lastname: Avanzini
authors.forename: Costanza
authors.initials: C
authors.lastname: Alvisi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Vanoli
authors.forename: Rachele
authors.initials: R
authors.lastname: Manca
authors.forename: Ombretta
authors.initials: O
authors.lastname: Luinetti
authors.forename: Franco
authors.initials: F
authors.lastname: Locatelli
authors.forename: Gino R
authors.initials: GR
authors.lastname: Corazza
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D005260:Female; D006801:Humans; D045164:Mesenchymal Stem Cell Transplantation; D008875:Middle Aged; D010288:Parenteral Nutrition; D016884:Polyendocrinopathies, Autoimmune / Q000188:drug therapy / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones