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Master Thesis

Entéropathies auto-immunes et auto-immunes like de l’adulte

Abstract : Objectives: Adult auto-immune enteropathy is a rare cause of diarrhea and malnutrion. The study aims to define clinical, immunological, histological and phenotypic features of adult auto-immune enteropathy, as well as efficient therapies. Methods : Medical files of 11 patients (from 3 French gastroenterological centers) were studied retrospectively. All the patients had diarrhea and intestinal villous atrophy from unknown origin, but were improved byimmunosuppressive treatment. Celiac disease and common variable immunodeficiency were excluded in all cases. Centralized analysis of biopsy specimens included histological review, and phenotypic analyses. Results : Mean age at onset of symptoms was 47 years, with 2 peaks : between 10 and 20, and after 60 years. Familial and personal history of auto-immune diseases was noted in 36% and 45% of the cases respectively. Anti AIE 75 kDa antibodies were found among more than half of the patients, and anti-enterocyte antibodies in 20% of the cases. Two patients with anti AIE 75 kDa antibodies and four patients with negative serology had been treated by Olmesartan at time of diagnosis. Intestinal villous atrophy, moderate increased levels of intra-epithelial lymphocytes and glandular apoptosis lesions were observed in all the patients. Lymphocytic gastritis or colitis was noted in 36% of patients. Except one patient, no abnormalities were observed in T reg counts but flow cytometry showed increased expression of CD57 and NKG2C on intestinal T lymphocytes. All the patients had a clinical response to steroids or immunosuppressor. Therapy targeting T cell response (anti TNF-a, ciclosporin, and thiopurines) appear more efficient than anti-B cell therapy. Two cases of T intestinal lymphoma (EATL and CD4+ T lymphoma) occurred during the follow-up, including one death. Conclusion : Auto-immune enteropathies with positive anti AIE 75 kDa serology, and auto-immune like enteropathies with negative serology show similar histological lesions with increased activated intestinal T lymphocytes. Use of Olmesartan was noted in 33% and 80% of seropositive and seronegative patients respectively, and appears as a triggering factor. Immunosuppressors were efficient, but 2 cases of lymphoma occurred during follow-up.
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Master Thesis
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  • HAL Id : dumas-01084183, version 1

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Sophie Scialom-Han. Entéropathies auto-immunes et auto-immunes like de l’adulte. Médecine humaine et pathologie. 2013. ⟨dumas-01084183⟩

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