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Treatment of FSGS with plasma exchange and immunadsorption
D Franke1, M Zimmering, N Wolfish
1Department of Pediatric Nephrology, Charité, Humboldt University at Berlin, Germany.
Insights
Plasma exchange (PE) and plasma immunadsorption (PIA) offer new treatment options for children with focal and segmental glomerulosclerosis (FSGS) resistant to standard therapies. Early intervention with PE or PIA can improve proteinuria remission rates in difficult-to-treat FSGS cases.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Immunology
Background:
- Focal and segmental glomerulosclerosis (FSGS) in children often has a poor prognosis without proteinuria remission.
- Current treatments like cyclosporine and steroids are effective in only about 60% of steroid-resistant cases, leaving a significant unmet need.
- Limited therapeutic options exist for children with cyclosporine-resistant primary FSGS.
Purpose of the Study:
- To evaluate the efficacy of plasma exchange (PE) and plasma immunadsorption (PIA) in children with steroid- and cyclosporine-resistant primary FSGS.
- To assess PE and PIA as salvage therapies for relapsing FSGS post-transplant and in native kidneys.
- To compare the effectiveness and resource demands of PE and PIA in treating resistant FSGS.
Main Methods:
- Nine children with cyclosporine-resistant primary FSGS were treated with PE.
- Seven patients with FSGS in native kidneys and two with relapsing FSGS post-transplantation also received PE.
- Patients unresponsive to PE or relapsing after PE were subsequently treated with PIA.
Main Results:
- Five of nine patients achieved complete remission of proteinuria with PE, and one achieved partial remission.
- Three patients did not respond to PE, often having a longer disease duration.
- PIA was effective in reducing proteinuria in patients who relapsed after PE cessation.
- PIA avoids the need for albumin or fresh-frozen plasma, unlike PE.
Conclusions:
- Plasma exchange and plasma immunadsorption are viable treatment options for pediatric FSGS resistant to conventional therapies.
- Early application of PE and PIA may lead to better outcomes in FSGS management.
- Plasma immunadsorption may be preferable to plasma exchange due to lower resource requirements and fewer complications.
Abstract:
In primary focal and segmental glomerulosclerosis (FSGS) renal prognosis is poor if no remission of proteinuria can be achieved with treatment. Currently, most children with FSGS are treated with cyclosporine and steroids after establishing steroid resistance, and approximately 60% of patients benefit from this therapy. For the remaining 40%, no generally approved therapeutic recommendations exist for children. We treated nine children with cyclosporine-resistant primary FSGS with plasma exchange (PE), two with relapsing FSGS after renal transplantation and seven with FSGS in their native kidneys. Three patients did not respond to PE, but five came into complete remission and one patient achieved partial remission. Three patients relapsed between 6 weeks and 2 years following cessation of PE, and were subsequently treated with plasma immunadsorption (PIA), which also reliably reduced proteinuria. The patients without response to PE tended to have a longer duration of the disease. We conclude that PE and PIA are a useful option for treatment 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 additional albumin or fresh-frozen plasma, as with PE.