The importance of perseverance in ANCA vasculitis

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I saw a patient in clinic in follow up this week who was interesting. A 90 year old thoroughly delightful woman who presented last July with acute renal failure, creatinine rising from normal to 4.4 within a period of weeks. Her Nephrologist in the community was on top of this quickly and in the context of an active urine sediment demonstrated a myeloperoxidase ANCA titer of 6400u. There is a natural inclination to question aggressive care delivery in patients of advanced age and this is appropriate in many circumstances as outcomes are often suboptimal. However, in this case our patient was functional, living independently, and as the record stated “appeared younger than her stated age”. So her referring Nephrologist decided to initiate dialysis for uremia and volume control and she was transferred to our facility for ongoing care.

We reviewed her options. She was already on dialysis. Her best/only hope of recovery involved a protocol of high dose solumedrol, oral cyclophosphamide, and based on best evidence a course of plasmapheresis. ANCA associated renal vasculitis is one of the few circumstance where we can offer patients the hope of recovery from dialysis dependency even when presenting with advanced stage disease. After discussion with her daughter our patient agreed to proceed and received three plasmapheresis exchanges, a gram of IV solumedrol over 3 days followed by an oral prednisone taper and oral cyclophosphamide loading dose and then daily dosing with a plan to continue for 6 months. She continued on dialysis and in truth had a difficult course with the usual complications associated with tolerating HD fluid shifts, fatigue and at least two hospital stays one of which was due to bacteremia and one for FUO. She required temporary discontinuation of cyclophosphamide after approximately four months due to persistent leukocytopenia and was switched to azathioprine which was discontinued during her admission with FUO in February.

Which brings me to the point of this blog. She was in to see me this week because her pre dialysis creatinine had fallen to 2.1mg% (from the mid 3 range) and her 24 hour urine collection returned a urea clearance of 6ml/min and creatinine clearance of 30ml/min. Her pANCA titer now 1.9units. So as of this week she has discontinued dialysis. She has maintained reasonable urine output over the duration and we are all hopeful that her days on dialysis are over. She still has a road ahead and she will likely have a significant component of CKD and she should be maintained on a some level of immunesuppression at least for now as relapse remains possible. She took delight in telling me that on the morning she saw me her usual dialysis transportation turned up (the message that she did not need them apparently not getting through) and she was able to greet them from her from door in her pj’s and send them on their way.

In the end this case illustrates the need to persevere with patients presenting with ANCA in the hope of gaining renal function recovery and understanding that pushing the age envelope sometimes turns out for the best.

This issue is addressed in this article by David Jayne of the EUVAS group.

Posted by David Steele M.D.


  1. So, how much po cytoxan loading dose did you guys give and how much maintenance? Also, what maintenance immunosuppression did you give her after she came off dialysis? thanks.

  2. Did you give an oral CYC loading dose as a single one-day dose?
    One-day oral CYC loading dose (for example, 500 mg) has been widely used in Hematolgy. In European Trials on AAV, we administered IV CYC. This is because a group from EUVAS recently showed that IV CYC may have some advantages in comparison to oral CYC.
    Notably, when we used an oral CYC loading dose, it was administered on a 3-days period. Daily oral CYC is administered during maintenance period.

  3. why don't? to persevere with this case

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