Effective treatment with rituximab for the maintenance of remission in frequently relapsing minimal change disease.

Eugenia Papakrivopoulou,Ali M Shendi,John O Connolly,Richard Trompeter,Alan D Salama,Maryam Khosravi

doi:10.1111/nep.12744

Eugenia Papakrivopoulou, Ali M Shendi + Show 4 more

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https://doi.org/10.1111/nep.12744

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Abstract

AimTreatment of frequently relapsing or steroid‐dependent minimal change disease (MCD) in children and adults remains challenging. Glucocorticoids and/or other immunosuppressive agents are the mainstay of treatment, but patients often experience toxicity from prolonged exposure and may either become treatment dependent and/or resistant. Increasing evidence suggests that rituximab (RTX) can be a useful alternative to standard immunosuppression and allow withdrawal of maintenance immunosuppressants; however, data on optimal treatment regimens, long‐term efficacy and safety are still limited.MethodsWe undertook a prospective study of RTX to allow immunosuppression minimization in 15 young adults with frequently relapsing or steroid‐dependent, biopsy‐proven MCD. All patients were in remission at the start of treatment and on a calcineurin inhibitor. Two doses of RTX (1 gr) were given 6 months apart. A subset of patients also received an additional dose 12 months later, in order to examine the benefit of re‐treatment. Biochemical and clinical parameters were monitored over an extended follow‐up period of up to 43 months.ResultsMedian steroid‐free survival after RTX was 25 months (range 4–34). Mean relapse frequency decreased from 2.60 ± 0.28 to 0.4 ± 0.19 (P < 0.001) after RTX. Seven relapses occurred, five of which (71%) when CD19 counts were greater than 100 µ. Immunoglobulin levels remained unchanged, and no major side effects were observed throughout the follow‐up period.ConclusionsRituximab therapy is effective at maintaining prolonged steroid‐free remission and reducing relapse frequency in this group of patients. Our study lends further support for the role of RTX in the treatment of patients with frequently relapsing or steroid‐dependent MCD.

Highlights

Aim: Treatment of frequently relapsing or steroid-dependent minimal change disease (MCD) in children and adults remains challenging
Steroid dependence was defined as relapse upon tapering steroid therapy or within 4 weeks of discontinuing steroids and need for long-term maintenance steroids 3 Maintenance immunosuppression with a calcineurin inhibitors (CNIs) 4 Remission of nephrotic syndrome (NS) defined as proteinuria
All patients were in remission and on a CNI at the time of RTX treatment

Summary

Introduction

Aim: Treatment of frequently relapsing or steroid-dependent minimal change disease (MCD) in children and adults remains challenging. Glucocorticoids and/or other immunosuppressive agents are the mainstay of treatment, but patients often experience toxicity from prolonged exposure and may either become treatment dependent and/or resistant. Methods: We undertook a prospective study of RTX to allow immunosuppression minimization in 15 young adults with frequently relapsing or steroid-dependent, biopsy-proven MCD. Two doses of RTX (1 gr) were given 6 months apart. A subset of patients received an additional dose 12 months later, in order to examine the benefit of re-treatment. Conclusions: Rituximab therapy is effective at maintaining prolonged steroidfree remission and reducing relapse frequency in this group of patients.

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