I have a 15yo girl with HD dependent ESRD over the last 2 years. We don’t know her underlying diagnosis, but we think she has Wegener's. She has no symptoms, other than pos p and c anca antibodies and recurrent severe jaw pain. A recent bone biopsy has just shown chronic inflammation, kidney biopsy at presentation showed 90% globally sclerosed glomeruli.
We were planning to list her for a renal tx last month, but she got very sick. We now know she had macrophage activation syndrome (ferritin 40000) and she has responded well to high dose steroids. Our Rheumatologist thinks it is her underlying autoimmune dz that provoked the macrophage activation, as her bone marrow biopsy is without signs of malignancy and she is EBV negative.
We are now trying to find someone with experience in doing solid organ tx after macrophage activation syndrome. How should we treat the macrophage activation syndrome, and when is she safe to undergo Tx?
We can’t seem to find anything in the literature.
An excellent review of the U.S. experience with renal transplant for WG: Outcomes of renal transplantation in recipients with Wegener's granulomatosis, Clin Transplant. 2011 May-Jun;25(3):380-387. Abstract at:
http://www.ncbi.nlm.nih.gov/pubmed/20394635
A cautionary note: MMF has been associated with exacerbation of acute inflammatory syndrome in case reports of 2 WG patients.
Full article at: http://ndt.oxfordjournals.org/content/17/5/923.full
Tough case this. I would have thought issues are:
i) Difficult to safely transplant if you're not genuinely sure of the diagnosis driving the MAS. If you can't rule out primary HLH on clinical features then maybe you should run a sCD25 assay?
ii) Treatment of MAS should include increased immunosuppression & ivIg
iii) As for when to transplant; the liver literature has a story of acute transplantation to ameliorate liver failure in the context of MAS, but it's difficult to see the analogy to kidney transplantation. If the ANCA diagnosis is secure then transplantation should be deferred until extrarenal remission has been complete for 12 months according to KDIGO guidelines.
http://www.bloodjournal.org/content/118/15/4041?sso-checked=true
http://www.ncbi.nlm.nih.gov/pubmed/23981123
My patient was transplanted just 6 weeks ago, and has done very well. After her MAS she was treated with pulse solumedrol x 3 doses, followed by oral steroids 60mg q day and MMF in a dose of about 1000mg/m sq /day. She became remarkably well after going on the this treatment. She had been ill on hemodialysis for months without any diagnosable cause. Oral Steroids were weaned completely as she was doing so well.
For the renal transplantation she was induced with Simulect and treated with Tacrolimus and MMF (our usual protocol), and she has been doing well except delayed graft function for 2 weeks. She is off dialysis now with almost normal renal function. Her MAS was not reactived during any of the surgeries.
Thanks for comments for my question
Very interesting case, thank you for shared it here. Tacrolimus was described as a treatment option for MAS (although mainly for secondary MAS, due to SLE).
I wonder how is she doing now?
She is doing very well, except being tired, as described above.She is looking foreward to starting school in 1 week. She has had 2 renal biopsies due to delayed graft function, both of which were normal.
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