Autoimmune enteropathy and villous atrophy in adults

Mesenchymal Stromal Cell Infusions as Rescue Therapy for Corticosteroid-Refractory Adult Autoimmune Enteropathy

“…18 When used in vivo, disappearance of pathogenic antibodies in an experimental model of autoimmune encephalomyelitis 19 and resolution of a case of antibody-mediated autoimmune thrombocytopenic purpura refractory to conventional therapies 20 have been reported. In the present patient, we observed increases, although transient, in intestinal plasma cell numbers and sIg levels with MSC infusions associated with (1) decreased circulating B cells, (2) decreased serum immunoglobulin levels, and (3) disappearance of pathogenic antibodies. Altogether, these findings seem to confirm the ability of MSCs to modulate B-cell function in vivo and to attenuate the autoimmune attack.…”

“…7 Therefore, AIE was investigated and confirmed by the presence of antienterocyte autoantibodies (Figure 1, A). 2 Since hospital admission, total parenteral nutrition, albumin supplementation, and corticosteroid therapy (methylprednisolone, 40 mg/d intravenously) had been given to maintain stable vital signs and electrolyte balance because of the persistence of abundant liquid stools. Treatment with autologous MSCs was, therefore, proposed as rescue therapy and was approved by the local bioethics committee.…”

“…1 Adult autoimmune enteropathy (AIE) is a rare disorder characterized by the presence of severe malabsorption syndrome, unresponsive to dietary restriction, whose diagnostic hallmarks are the positivity of antienterocyte autoantibodies and the presence of villous atrophy with inflammatory infiltration of the small bowel mucosa, indistinguishable from that of celiac disease. 2 Although the understanding of the pathogenesis of AIE has greatly improved in recent years, 3,4 treatment is still not standardized, being mainly based on immunosuppressive or biological therapy and parenteral nutrition. 5 In view of the successful use of MSCs for the treatment of AIE in a mouse model of multiorgan autoimmunity, 6 we used this treatment as rescue therapy in a patient with AIE and life-threatening malabsorption syndrome; we also performed ancillary immunologic studies to gain insights into the mechanisms at the basis of MSC efficacy in vivo.…”

Olmesartan-associated enteropathy: results of a national survey

“…Clinical, biological and histological manifestations All 32 patients (100%) had diarrhoea, with a median number of 8 liquid stools per day [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Twenty-four patients (75%) had abdominal pain, which was rarely severe (n = 3).…”

Celiac Disease and Autoimmunity in the Gut and Elsewhere

“…One of the initial reports of adult-onset autoimmune enteropathy was in 1997 from Corazza et al describing two of four patients with DQ2 HLA typing, clinically non-responsive to gluten restriction, and who were found to be positive for anti-enterocyte antibodies. [67] Additional reported cases of adults with AIE or AIE variants include: AIE associated with atrophic gastritis and colitis, autoimmune enterocolitis, AIE in association with systemic autoimmune disease (sicca syndrome, thyroiditis), and AIE associated with rheumatoid arthritis. The pathophysiology of AIE is unclear.…”