Tuesday, September 14, 2010


A 87 y old patient comes in with ANCA negative Active RPGN, biopsy confirms pauci immune RPGN. Patient's presenting crt is 5.0mg/dl and quickly rises to require hemodialysis? What induction treatment would you choose?
Oral Cyclophosphamide + steroids + plasmapheresis  10%
IV Cyclophosphamide + steroids + plasmapheresis 68%
Steroids only  4%
Rituximab + steroids+ plasmapheresis 2%
Rituximab + steroids only  0%
Mycophenolate + steroids 2%
Do not treat as age is limiting factor 12%

Interesting break down. First and foremost. This is a tough situation. Although the age group where we see ANCA vasculitis is in the 65+ age group, as the age advances, the question always comes up- is the treatment more harmful than the disease?
I think that there were a fair number 12% that didn't want to treat. I think thats reasonable.
Now, the data on ANCA+ vasculitis is there but not that great. Usually most studies have used PO cytoxan and not IV cytoxan and recently two trials in the NEJM 2010 compared IV cytoxan in one trial to Rituximab and other PO cytoxan with rituximab. One can postulate that IV cytoxan would work as well.  I think that both IV and PO cytoxan would be ideal answers along with Pheresis and steroids.  I was surprised that not many of you chose rituximab given the latest findings. There is just observational data on MMF in this disease.

Now, all the data I have been talking about are in ANCA+ vasculitis.  The case above was an ANCA negative pauci immune vasculitis.  You can guess how much data is on that disease.  Most of us would treat very similar to ANCA + vasculitis and possible remove any bad humor that is causing this vasculitis, perhaps its LAMP-2 antibodies some might say.  Some might call it Atypical ANCA.  20-30% of microscopic polyangitis and Wegener's is ANCA negative. The treatment probably should remain the same, but the data is skimpy!
Here are some prior posts from us on this regard:

1 comment:

  1. I would like to add the Cyclops trial data here. I am sorry I forgot to talk about that here. This trial actually compared ANCA positive patients randomly PO cytoxan vs IV cytoxan and found that the cumulative dose of cytoxan was 50% less in the IV group. Relapse rates were similar and leukopenia was less in IV group. So based on that trial, IV cytoxan should be the treatment of choice. Hence what you all chose.
    But in ANCA negative vasculitis, no data available and probably can extrapolate from this trial.


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