Extracorporeal plasma treatment in primary and recurrent focal segmental glomerular sclerosis: A review

This review summarizes the state of the art of apheresis in focal segmental sclerosing glomerulonephritis (FSGS) based on the current literature. FSGS is a major cause of nephrotic syndrome and renal insufficiency and has a high tendency to recur after renal transplantation. In many instances. treatment with steroids and/or immunosuppressive agents is without effect. Therapeutic plasma exchange (as well as immunoadsorption in some instances) was performed in primary (pFSGS) and recurrent (rFSGS) FSGS. Proteinuria could be improved and renal function was stabilized in 58% of rFSGS adults, in 74% of rFSGS children, and in 44% of pFSGS in adults. Treatment was successful if implemented early, i.e.. in the stage of minimal change glomerulopathy before significant sclerosis had developed. This approach will probably be realistic only in the regular follow-up after transplantation. Furthermore. some patients with full-blown pFSGS were treated successfully in a prophylactic indication immediately prior to transplantation which decreased the incidence of recurrence to 26% in treated patients versus 54% in controls. Due to the uncontrolled study design and small patient cohorts in most trials. the major disadvantage of the reports currently available on the use of apheresis in FSGS is their low level of evidence from which the previous conclusions had to be drawn. Large prospective. controlled and randomized clinical trials are urgently needed for recommendations based on high-level evidence.