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Mol Immunol. 2011 Aug;48(14):1604-10. doi: 10.1016/j.molimm.2011.04.005. Epub 2011 May 24.

Dense deposit disease.

Author information

  • 1Department of Internal Medicine, Division of Nephrology, Carver College of Medicine, University of Iowa, 21151 PFP, 200 Hawkins Drive, Iowa City, IA 52242, USA. Richard-smith@uiowa.edu

Abstract

Dense deposit disease (DDD) is an orphan disease that primarily affects children and young adults without sexual predilection. Studies of its pathophysiology have shown conclusively that it is caused by fluid-phase dysregulation of the alternative pathway of complement, however the role played by genetics and autoantibodies like C3 nephritic factors must be more thoroughly defined if we are to make an impact in the clinical management of this disease. There are currently no mechanism-directed therapies to offer affected patients, half of whom progress to end stage renal failure disease within 10 years of diagnosis. Transplant recipients face the dim prospect of disease recurrence in their allografts, half of which ultimately fail. More detailed genetic and complement studies of DDD patients may make it possible to identify protective factors prognostic for naïve kidney and transplant survival, or conversely risk factors associated with progression to renal failure and allograft loss. The pathophysiology of DDD suggests that a number of different treatments warrant consideration. As advances are made in these areas, there will be a need to increase healthcare provider awareness of DDD by making resources available to clinicians to optimize care for DDD patients.

Copyright © 2011 Elsevier Ltd. All rights reserved.

PMID:
21601923
[PubMed - indexed for MEDLINE]
PMCID:
PMC3142282
Free PMC Article
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