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An update on the pathogenesis and treatment of IgA nephropathy.
MedLine Citation:
PMID:  22318424     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Over the past two decades significant progress has been made in unravelling the complex pathogenesis of immunoglobulin A nephropathy (IgAN). Excess amounts of poorly galactosylated immunoglobulin (Ig)A1 in the serum appear to be the trigger for generation of glycan-specific IgG and IgA autoantibodies, resulting in the formation of circulating IgA immune complexes, which are pivotal to the development of nephritis. It remains unclear why there is an increase in poorly galactosylated IgA1 molecules in the serum in IgAN. One intriguing possibility is that this IgA is derived from displaced mucosal B cells, which have mis-homed from their mucosal induction sites to systemic sites, where they secrete polymeric, poorly galactosylated IgA directly into the circulation rather than onto mucosal surfaces. Lack of a clear appreciation of the origins of poorly galactosylated IgA1 and an incomplete understanding of immune complex formation have hampered development of specific therapeutic strategies to prevent mesangial IgA deposition. Clinicians have therefore been left to manage patients with generic therapies, mainly by control of blood pressure and renin-angiotensin blockade. A paucity of high-quality clinical trials has meant that evaluation of additional therapies, particularly immunosuppressive regimens, has been difficult and there remains a great deal of confusion over the optimum treatment of patients at high risk of progressive chronic kidney disease.Kidney International advance online publication, 8 February 2012; doi:10.1038/ki.2011.501.
Authors:
Joanna K Boyd; Chee K Cheung; Karen Molyneux; John Feehally; Jonathan Barratt
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-2-08
Journal Detail:
Title:  Kidney international     Volume:  -     ISSN:  1523-1755     ISO Abbreviation:  -     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-2-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0323470     Medline TA:  Kidney Int     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
1] The John Walls Renal Unit, Leicester General Hospital, Leicester, UK [2] Department of Infection, Immunity and Inflammation, University of Leicester, Leicester, UK.
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