Autoimmunity in Wiskott-Aldrich syndrome

Details

Serval ID
serval:BIB_D46D86B0E405
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Title
Autoimmunity in Wiskott-Aldrich syndrome
Journal
Curr Opin Rheumatol
Author(s)
Schurman S. H., Candotti F.
ISSN
1040-8711 (Print)
ISSN-L
1040-8711
Publication state
Published
Issued date
07/2003
Volume
15
Number
4
Pages
446-53
Language
english
Notes
Schurman, Shepherd H
Candotti, Fabio
eng
Review
Curr Opin Rheumatol. 2003 Jul;15(4):446-53.
Abstract
As many as 40% of patients with Wiskott-Aldrich syndrome may eventually suffer from an autoimmune disorder, with an increased chance of developing a malignancy. Vasculitides and autoimmune hemolytic anemia are the two most common autoimmune manifestations and often cause considerable morbidity and mortality, because they may require treatment with bone marrow transplantation. Insights into the mechanisms of autoimmunity have provided clues to the pathogenesis of these disorders in Wiskott-Aldrich syndrome. Chronic inflammation, interleukin-2 deficiency, and increased apoptosis may all play a possible role in the loss of peripheral tolerance to self-antigens in this disease. This article reviews the manifestations and consequences of autoimmunity in Wiskott-Aldrich syndrome, its possible mechanisms, and available treatments.
Keywords
Autoimmune Diseases/*genetics/pathology, Autoimmunity/*immunology, Humans, Wiskott-Aldrich Syndrome/*immunology/pathology
Pubmed
Create date
01/11/2017 10:29
Last modification date
20/08/2019 15:54
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