Management of severe refractory adult autoimmune enteropathy with infliximab and tacrolimus.
BACKGROUND
Autoimmune enteropathy (AIE) is a rare cause of intractable diarrhea in infancy. A variant with onset in adulthood has recently been described. The immune mechanisms underlying AIE are unclear and the experience in the management of adult AIE is very limited.


METHODS
A 19-year-old male patient with massive refractory diarrhea and significant weight loss was diagnosed with AIE. He did not improve with corticosteroid and azathioprine treatment and was then treated successfully with infliximab. The response to treatment was rapid and complete. Because of a severe life-threatening hypersensitivity reaction during the 4th infliximab dose, infliximab had to be discontinued and therapy with tacrolimus was initiated. A complete clinical remission has since been maintained.


CONCLUSIONS
Both infliximab and tacrolimus may provide a therapeutic alternative in patients with adult refractory AIE.
von Hahn
Thomas
T
Department of Internal Medicine, Divisions of Gastroenterology and Hepatology, Charité Medical School-Campus Virchow, Augustenburger Platz 1, DE-13353 Berlin, Germany.
Stopik
Dieter
D
Koch
Martin
M
Wiedenmann
Bertram
B
Dignass
Axel
A
D000284:Administration, Oral; D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000473:pathology; D001706:Biopsy; D004359:Drug Therapy, Combination; D016099:Endoscopy, Gastrointestinal; D005500:Follow-Up Studies; D005765:Gastrointestinal Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000069285:Infliximab; D007262:Infusions, Intravenous; D007410:Intestinal Diseases / Q000188:drug therapy* / Q000473:pathology; D007413:Intestinal Mucosa / Q000473:pathology; D008297:Male; D012720:Severity of Illness Index; D016559:Tacrolimus / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D005765:Gastrointestinal Agents; D007166:Immunosuppressive Agents; D000069285:Infliximab; D016559:Tacrolimus