Sunday, May 11, 2008

Dense Deposit Disease: MPGN Type II No Longer?

The classical teaching about Dense Deposit Disease (DDD) usually begins with its classification as type II Membranoproliferative Disease.

However, in the most recent edition of the NephSAP "Glomerular, Vascular, and Tubulointerstitial Diseases" module, the case is made that Dense Deposit Disease should no longer be considered as a MPGN.

In a recent review of 69 DDD biopsy specimens, only about 28% of cases demonstrated MPGN histology; the majority (about 50% of cases) actually showed a mesangioproliferative lesion.

In contrast to MPGN Type I (which is often associated with hepatitis C, other chronic infections, or autoimmune disease), DDD is predominantly a pediatric disease that presents as nephritic syndrome. The abnormal irregular GBM deposits appear to arise as a result of unregulated activation of the alternative pathway of complement deposition. Most commonly this results from the presence of C3 nephritic factor (a circulating, activating antibody against C3 convertase) though it can also occur with hereditary factor H Deficiency.

3 comments:

Amanda said...

Hello, I found your blog while searching for dense deposit disease, I am a 2nd year renal fellow at vanderbilt and I really enjoyed your blog! Great work AND dedication. good luck

Anonymous said...

hello i have mpgn type 2 and it is quite the roller coaster of symptoms. if you have questions or info that would be great. my email is seymourforyou@msn.com

Mike & Chris said...

My wife is a patient at Columbia Presbyterian Hospital in NYC and she has been diagnosed with both dense deposit (before and after her kidney transplant of 4 months ago) and now the team has added a diagnosis of RPGN. Has anyone seen these 2 diagnoses in the same patient before? She has been in the hospital now for 28 days and my 3 children and I would like to have her home, especially since we live 3 hours away from the hospital. Please email mckrauss@stny.rr.com with any info or questions. Thanks, Mike