Long-Term Outcomes of Kidney Transplant Recipients With Primary Idiopathic Focal Segmental Glomerulosclerosis.

Background: Few data exist on recurrence rates, treatment response, and long-term outcomes in kidney transplant recipients (KTR) with primary focal segmental glomerulosclerosis (FSGS).
Methods: This retrospective, observational study included 1218 consecutive KTR during 2002 to 2016. All patients with primary idiopathic FSGS were identified through application of strict diagnostic criteria. Outcomes were followed over an average of 70.4 months.
Results: We identified 48 KTR (3.9%) with primary FSGS. Seven-year death-censored graft survival rate was 81% (primary FSGS) versus 85% (control) (P = .297). Eighteen KTR had FSGS recurrence (predicted incidence, 50% after 7 years). Seven-year death-censored graft survival rate in KTR with FSGS recurrence was significantly worse than in FSGS KTR without recurrence (63% versus 96%, P = .010). In the case of FSGS recurrence, a multi-modal treatment approach was applied, including plasma exchange (PE) (100% of patients), intravenous cyclosporine (50%), rituximab (61%), and the "Multiple Target Treatment" (39%). The median number of PE sessions was 27. Proteinuria decreased significantly and persistently during the course of treatment. Complete remission of FSGS was observed in 7 patients (39%); another 7 patients (39%) had partial remission (PE dependence was observed in 4 patients [22%]). Four patients (22%) with FSGS recurrence had early graft loss (<6 months after transplant) despite all treatment efforts.
Conclusions: In KTR with primary FSGS, a high proportion of recurrence occurred, and recurrence was associated with significantly worse death-censored graft survival rates. However, a multi-modal treatment approach led to improvement of proteinuria and full or partial remission in most patients. Importantly, overall death-censored graft survival rate in KTR with primary FSGS was comparable with that in the control group.
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