Adult-Onset Autoimmune Enteropathy in an European Tertiary Referral Center.
Adult
Aged
Atrophy
Autoantibodies
/ analysis
Chronic Disease
Diarrhea
/ etiology
Duodenum
/ pathology
Enterocytes
/ immunology
Fatty Acid-Binding Proteins
/ blood
Female
HLA-DQ Antigens
/ blood
Humans
Immunosuppressive Agents
/ therapeutic use
Male
Middle Aged
Netherlands
Parenteral Nutrition
Polyendocrinopathies, Autoimmune
/ diagnosis
Retrospective Studies
T-Lymphocyte Subsets
/ metabolism
Tertiary Care Centers
Young Adult
Journal
Clinical and translational gastroenterology
ISSN: 2155-384X
Titre abrégé: Clin Transl Gastroenterol
Pays: United States
ID NLM: 101532142
Informations de publication
Date de publication:
01 08 2021
01 08 2021
Historique:
received:
02
02
2021
accepted:
28
06
2021
entrez:
1
8
2021
pubmed:
2
8
2021
medline:
19
1
2022
Statut:
epublish
Résumé
Adult-onset autoimmune enteropathy (AIE) is a rare cause of severe chronic diarrhea because of small intestinal villous atrophy. We report on patients with adult-onset AIE in an European referral center. Retrospective study including patients diagnosed with AIE in the Amsterdam UMC, location VUmc, between January 2003 and December 2019. Clinical, serological, and histological features and response to treatment were reported. The specificity of antienterocyte antibodies (AEA) was evaluated by examining the prevalence of AEA in (i) controls (n = 30) and in patients with (ii) AIE (n = 13), (iii) celiac disease (CD, n = 52), (iv) refractory celiac disease type 2 (n = 18), and (v) enteropathy-associated T-cell lymphoma (EATL, n = 10). Thirteen AIE patients were included, 8 women (62%), median age of 52 years (range 23-73), and 6 (46%) with an autoimmune disease. AEA were observed in 11 cases (85%), but were also found in CD (7.7%), refractory celiac disease type 2 (16.7%), and EATL (20%). Ten patients (77%) were human leukocyte antigen DQ2.5 heterozygous. Total parenteral nutrition was required in 8 cases (62%). Steroids induced clinical remission in 8 cases (62%). Step-up therapy with rituximab, cyclosporine, infliximab, and cladribine in steroid-refractory patients was only moderately effective. Four patients died (31%), but 4 (31%) others are in long-term drug-free remission after receiving immunosuppressive treatment, including 1 patient who underwent autologous stem cell transplantation. Adult-onset AIE is a rare but severe enteropathy that occurs in patients susceptible for autoimmune disease. Four patients (31%) died secondary to therapy-refractory malabsorption, while immunosuppressive therapy leads to a long-lasting drug-free remission in one-third of patients.
Identifiants
pubmed: 34333499
doi: 10.14309/ctg.0000000000000387
pii: 01720094-202108000-00003
pmc: PMC8323799
doi:
Substances chimiques
Autoantibodies
0
Fatty Acid-Binding Proteins
0
HLA-DQ Antigens
0
Immunosuppressive Agents
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e00387Informations de copyright
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.
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