Tuesday, November 15, 2011

ASN2011: Complement related disorders

This ASN, there was a series of talks regarding complement glomerulopathy and the use of certain newer agents for treatment. Dr Licht discussed this in detail in one of the talks.

Bottom line

1. MPGN pattern of injury is seen in complement related glomerular diseases.
2. DDD is now part of C3 glomerulopathies
3. Complement factor H antibodies, complement factor B antibodies have been associated with C3 glomerular disease since they will enhance c3 conversion and eventually affect the alternate pathway.
4. Genetic forms such as mutations in Factor H, CFHR5, C3 polymorphisms were also noted in that case.
5. It is possible that what we used to call perhaps C3 only post infectious GN or resolving post infectious GN was really C3 glomerulopathy.
6. Treatment is plasma exchange as there might be antibody that exists ( if you think there is)
7. Complement inhibition is the key- and the only drug we have is eculizumab ( 4 doses 900mg IV per week and 1200mg per week following that for 4 doses):- but expensive
8. Overall, there is a paradigm shift happening in introduction of these disorders.

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