Dense deposit disease: new insights.
OBJECTIVE
Dense deposit disease is a rare but devastating disease primarily affecting children. This review focuses on new information regarding the pathophysiology of dense deposit disease, its appearance histopathologically, its relationship to other diseases including macular degeneration and acquired partial lipodystrophy and potential new therapies.


RESULTS
The microscopic features of dense deposit disease have been separated into five patterns with only about 25% of patients showing membranoproliferative features. The subtle interplay between genetic changes in complement regulatory proteins and dysregulation of the alternative pathway of complement is now more evident. Haplotype mapping has shown at-risk phenotypes of complement factor H associated with the development of dense deposit disease. Treatment protocols are empiric and not very effective. New information on complement inhibitors and plasma exchange, however, has brought hope for new therapies in the near future.


CONCLUSIONS
Understanding of the pathology and the pathophysiology of dense deposit disease has advanced rapidly in the last decade. New efforts in genetic mapping along with the development of novel inhibitors of the complement system will lead to improved care for patients afflicted with this uncommon condition.
Walker
Patrick D
PD
Nephropathology Associates, Little Rock, Akansas 72211, USA. walkerpd@kidneybx.com
D000818:Animals; D017242:Complement Factor H / Q000276:immunology; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D003170:Complement Pathway, Alternative / Q000235:genetics; D003165:Complement System Proteins / Q000235:genetics / Q000276:immunology*; D020022:Genetic Predisposition to Disease; D050533:Glomerular Basement Membrane / Q000473:pathology*; D015432:Glomerulonephritis, Membranoproliferative / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D016030:Kidney Transplantation; D008060:Lipodystrophy / Q000276:immunology; D008268:Macular Degeneration / Q000276:immunology
D051056:Complement Inactivating Agents; D017242:Complement Factor H; D003165:Complement System Proteins