Treatment of FSGS with plasma exchange and immunadsorption

In primary focal and segmental glomerulosclerosis (FSGS) renal prognosis is poor if no remission of proteinuria can be achieved with treatment. Curren tly, most children with FSGS are treated with cyclosporine and steroids aft er establishing steroid resistance, and approximately 60% of patients benef it from this therapy. For the remaining 40%, no generally approved therapeu tic recommendations exist for children. We treated nine children with cyclo sporine-resistant primary FSGS with plasma exchange (PE), two with relapsin g FSGS after renal transplantation and seven with FSGS in their native kidn eys. Three patients did not respond to PE, but five came into complete remi ssion and one patient achieved partial remission. Three patients relapsed b etween 6 weeks and 2 years following cessation of PE, and were subsequently treated with plasma immunadsorption (PIA), which also reliably reduced pro teinuria. The patients without response to PE tended to have a longer durat ion of the disease. We conclude that PE and PIA are a useful option for tre atment of steroid- and cyclosporine-resistant FSGS, particularly if applied early in the course of the disease. Although more demanding on supportive resources, PIA seems preferable to PE, since there is no necessity for addi tional albumin or fresh-frozen plasma, as with PE.