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Wednesday, January 13, 2010

TOPIC DISCUSSION: POST TRANSPLANT RECURRENCE FSGS


Recurrence in the allograft of FSGS is devastating both to the patient and the physicians taking care of such patients. The time frame is variable but its usually rapid and usually first signs of it might be minimal change on biopsy and not full blown FSGS.  Its thought to be secondary to a circulatory factor or the absence of normally present factor in plasma, each causing podocytopathy.

THINGS TO TAKE HOME:
1. Rule out viral causes:- HIV, parvovirus b19 infection , CMV, EBV and Hepatitis B and C but usually this is seen with collapsing glomerulopathy.
(usually seen with low immunoglobulin levels)
2. Rule out familial FSGS, usually mutations in ACTN4, NPHS2, TRPC6, and CD2AP, especially if donor was a living related person.
3. Drug induced--> classically sirolimus causing a both collapsing GN and a podocytopenic FSGS

TREATMENT options:
1. Plasmapheresis:- Initial studies showed transient benefit after several weeks.  Best results are consecutive treatements for 3 days for total of 9 treatments and then spacing them out. 1.5 Plasma volume is what is required.  How long to continue this treatment and how to space it out is anecdotal.  No randomized studies have been done with this treatment
2. IVIG:- might be an option if there is viral induced and there is hypogammaglobulinemia.
3. Plasmapheresis with Cytoxan:- 2 studies with some benefit noted. In my opinion, already on triple regimen for transplant, what is the benefit of Cytoxan?
4. Plasmapheresis with CNI:- some evidence but by default most patients are getting this anyway as they are post transplant, again case reports and series
5. Steroids:- keep higher dose post transplant:- case reports and series
6. Rituximab:- few case reports with variable success, only worked 50% of the time
7. Galactose:- some emerging data that IV form of this sugar and possible PO might be beneficial in treating resistant FSGS. No definite data yet.

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