Journal article
Dense deposit disease
Molecular immunology, Vol.48(14), pp.1604-1610
2011
DOI: 10.1016/j.molimm.2011.04.005
PMCID: PMC3142282
PMID: 21601923
Abstract
► Dense deposit disease (DDD) is a rare complement-mediated renal disease. ► DDD is caused by fluid-phase dysregulation of the alternative pathway of complement. ► Half of DDD patients progress to end-stage renal failure. ► No mechanism-directed therapies are currently available. ► Genetic and complement studies are needed to identify factors prognostic for outcome.
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.
Details
- Title: Subtitle
- Dense deposit disease
- Creators
- Richard J.H Smith - Department of Internal Medicine, Division of Nephrology and Department of Otolaryngology, Carver College of Medicine, University of Iowa, 21151 PFP, 200 Hawkins Drive, Iowa City, IA 52242, USAClaire L Harris - Department of Infection, Immunity, and Biochemistry, School of Medicine, Cardiff University, Cardiff, UKMatthew C Pickering - Centre for Complement and Inflammation Research, Division of Immunology and Inflammation, Faculty of Medicine, Imperial College, London, UK
- Resource Type
- Journal article
- Publication Details
- Molecular immunology, Vol.48(14), pp.1604-1610
- DOI
- 10.1016/j.molimm.2011.04.005
- PMID
- 21601923
- PMCID
- PMC3142282
- NLM abbreviation
- Mol Immunol
- ISSN
- 0161-5890
- eISSN
- 1872-9142
- Publisher
- Elsevier Ltd
- Language
- English
- Date published
- 2011
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984006412002771
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