Abstract
BackgroundSteroid-resistant nephrotic syndrome (SRNS), commonly caused by focal segmental glomerulosclerosis (FSGS), is associated with progression to stage 5 chronic kidney disease, requirement for kidney replacement therapy and a risk of disease recurrence post-kidney transplantation. Ofatumumab (OFA) is a fully humanised monoclonal antibody to CD20, with similar mechanisms of action to rituximab (RTX).MethodsWe report a case series of seven UK patients (five paediatric, two adult), all of whom developed FSGS recurrence after kidney transplantation and received OFA as part of their therapeutic intervention. All also received concomitant plasmapheresis. The 2-year outcome of these seven patients is reported, describing clinical course, kidney function and proteinuria.ResultsFour patients (all paediatric) achieved complete urinary remission with minimal proteinuria 12 months post-treatment. Three of those four also had normal graft function. Two patients showed partial remission—brief improvement to non-nephrotic proteinuria (197 mg/mmol) in one patient, maintained improvement in kidney function (estimated glomerular filtration rate 76 ml/min/1.73 m2) in the other. One patient did not demonstrate any response.ConclusionsOFA may represent a useful addition to therapeutic options in the management of FSGS recurrence post-transplantation, including where RTX has shown no benefit. Concomitant plasmapheresis in all patients prevents any definitive conclusion that OFA was the beneficial intervention.
Highlights
Steroid-resistant nephrotic syndrome (SRNS) is defined as proteinuria > 200 mg/mmol creatinine, hypoalbuminaemia and clinical oedema with no remission of proteinuria following 4 weeks of high-dose (2 mg/kg) corticosteroids, or subsequent loss of steroid responsiveness over time
Stage 5 chronic kidney disease (CKD 5) and requirement for kidney replacement therapy is common in focal segmental glomerulosclerosis (FSGS)—43–94% of patients enter CKD 5 within 10 years of diagnosis, depending on immunosuppressant response [1]
Various immunosuppressive agents trialled in FSGS include calcineurin inhibitors, mycophenolate mofetil and the chimeric anti-CD20 monoclonal antibody, rituximab (RTX); calcineurin inhibition is recommended as first-line treatment in pre-dialysis patients
Summary
Steroid-resistant nephrotic syndrome (SRNS) is defined as proteinuria > 200 mg/mmol creatinine, hypoalbuminaemia and clinical oedema with no remission of proteinuria following 4 weeks of high-dose (2 mg/kg) corticosteroids, or subsequent loss of steroid responsiveness over time. Steroid-resistant nephrotic syndrome (SRNS), commonly caused by focal segmental glomerulosclerosis (FSGS), is associated with progression to stage 5 chronic kidney disease, requirement for kidney replacement therapy and a risk of disease recurrence post-kidney transplantation. Methods We report a case series of seven UK patients (five paediatric, two adult), all of whom developed FSGS recurrence after kidney transplantation and received OFA as part of their therapeutic intervention. Results Four patients (all paediatric) achieved complete urinary remission with minimal proteinuria 12 months post-treatment. Three of those four had normal graft function. Conclusions OFA may represent a useful addition to therapeutic options in the management of FSGS recurrence post-transplantation, including where RTX has shown no benefit. Concomitant plasmapheresis in all patients prevents any definitive conclusion that OFA was the beneficial intervention
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