ESPN 50th Annual Meeting

ESPN 2017


 
LONG-TERM OUTCOME OF KIDNEY TRANSPLANTATION IN CHILDREN WITH FSGS
JASNA SLAVIčEK KRISTINA VRLJIčAK DANKO MILOšEVIć MAJA LEMAC 1

1- UHC Zagreb, Zagreb, Croatia
2- UHC Zagreb, Zagreb, Croatia
 
Introduction:

Recurrence of  focal segmental glomerulosclerosis (FSGS) occures in aproxymatelly 30% of patients after renal transplantation (Tx) and  negatively impacts graft survival. Risk faktorsfor recurrence are age >6 years <  15 years, presence of mesangial hyper cellularity. at onset of nephrotic  syndrome and rapid progression of the disease  to ESRD in less than 3 years  in the native kidneys. Retransplantation of a recipient with  recurrence  of FSGS in a perevious graft brings 80 % risk a recurrence rate. Therapeutic options  to produce a remission following recurrence  of NS include  Plasmaexchange ( PE), high dose of cyclosporine, PE  and cyclophosphamide, Plasma protein absorption, and recently  rituximab, but there were not control studies.

Material and methods:

Medical  records  from 121 kidney transplanted (kTx) children from 1982-2016 were collected  and analysed. Most of them  were transplanted in our country, some in abrod (France, Austria, Italy). In 17/121 ( 20,5%) primary renal disease  were FSGS. In all  patients  FSGS as original diesease and  as a  recurrence  was biopsy proven. Three pts received  second graft. One  of them had FSGS  recurrence in the second graft  as well as the first.

The patient  with mesangioprolipherative GN  in his  native kidneys  developed  massive proteinuria  on the  first day after Tx. Graft  biopsy showed FSGS.

Imunosupression was CyA, Aza, Steroids earlier and CyA or tacrolimus, MMF, Steroids recently.

Plasmaexchange ( PE)  was preformed in 3  pts  with FSGS recurrence and rituximab  with PE in 2. In Patient  with HBV infection  only steroid boluses  were administered.

 

 

Results:

Age  ys                                                         11,46 ± 7,2

Follow up   ys                                                 6,8   ± 21,2

Source of  graft   DD/LD                              14/3

Retransplanted- second graft                        1

Age at onset NS                                             6,1 ±  7,4        

Reccurence of FSGS                                     5/17

Onset of  proteinuria after Tx                         1day-7 mos

Treatment modality:                                       PE, ACE, Rituximab, high doses of methylprednisolone     

Outcome:                                                        Graft loss after 1 months - 3,5 yrs. Complete remissIon in one                                                                                                after preemptive Rituximab and PE

 

 

 

Conclusions:

Recurrence of FSGS after kTx is associated with impared  graft function  and graft loss. Current therapy with PE and rituximab is not  always effective. We neeed more controlled studies  to answer the issue  when to start  therapy, which therapy and how long.