Emerging treatments
Plasmapheresis
Plasmapheresis is useful for recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation.[53][54] However, managing FSGS in the context of renal transplantation remains controversial.
Rituximab
Rituximab is a chimeric monoclonal antibody directed against the CD20 antigen on B cells. Treatment with rituximab has been successful in patients with B-cell lymphomas and autoimmune diseases. Observational studies have suggested rituximab may be effective in treating patients with minimal change nephropathy and FSGS.[55] Four patients with nephrotic syndrome due to minimal change nephropathy or FSGS were treated with rituximab because of failure of or intolerance to the standard immunosuppressive therapy.[56] Other cases of FSGS reported in the literature (6 paediatric patients) that were successfully treated with rituximab were also included. Complete remission was reported within 1 month for a 7-year-old boy with FSGS during treatment with rituximab and concurrent treatment with mycophenolate, low-dose prednisolone, and tacrolimus.[56] Controlled trials are needed to further evaluate the efficacy of rituximab in FSGS.
Pirfenidone
Pirfenidone has been shown to have therapeutic potential in fibrotic diseases, although the mechanism of action is not well understood. It has been shown to reduce transforming growth factor-beta 1 production, antagonise tumour necrosis factor-alpha signalling, and scavenge reactive oxygen species. It also reduces fibrosis and prevents loss of glomerular filtration in animal models of renal disease. An open-label trial evaluated the safety and efficacy of pirfenidone in patients with idiopathic and post-adaptive FSGS.[57] Pirfenidone had no effect on blood pressure or proteinuria but it did preserve renal function. Controlled trials are needed to further evaluate the efficacy of pirfenidone in FSGS.
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