Abstract
Minimal change disease (MCD) accounts for 15% of adult nephrotic syndrome (NS) cases. Adult-MCD patients may have more severe clinical features than pediatric patients. In children, Rituximab (RTX) has been used since 2006 to treat frequently relapsing NS. In adults, data about the efficacy of RTX for MCD are limited. We report our experience on the use of RTX in adult biopsy-proven MCD. Our series includes 6 adult patients (2 males and 4 females), age 45–73 years, treated with RTX (4 weekly doses of 375 mg/m2). Proteinuria decreased from 11,2 (23–4.8) g/24 hours to 0.6 (0–2) g/24 hours after 6 months, and to 0.4 (0–1, 4) g/24 h in the 4 pts with the longer follow-up. Creatinine decreased from 1.95 (0.5–5) mg/dl to 0.88 (0.6–1.3) mg/l. Five patients achieved a complete renal remission, while in 1 pt proteinuria decreased by 75%. RTX successfully depleted CD19 lymphocytes in 100% of pts for at least 6 months. No clinically relevant adverse events have been observed. This case series shows a remarkable efficacy of RTX in treatment of MCD. RTX can be an attractive alternative both in recurrent forms and in induction-therapy of MCD. RTX may be preferentially used in patients at a high risk of development of the adverse effects of corticosteroids and should be considered as an alternative option in patients with recurrent NS. Additional data are needed to inform clinical practice on how best to use RTX in this patient population, so that definitive randomized trials can be planned.
Highlights
Minimal change disease accounts for 15% of adult cases of nephrotic syndrome [1].Because of its high prevalence in children, most studies on the natural course, treatment, and prognosis of MCD have focused on pediatric population
Minimal change disease (MCD) accounts for 15% of adult nephrotic syndrome (NS) cases
All pts were treated with RTX as first-line therapy
Summary
Minimal change disease accounts for 15% of adult cases of nephrotic syndrome [1].Because of its high prevalence in children, most studies on the natural course, treatment, and prognosis of MCD have focused on pediatric population. Adult-onset MCD patients (pts), especially those who are found to have focal segmental glomerulosclerosis (FSGS) on a second kidney biopsy, might experience a progression to ESRD [3]. Adult-onset MCD patients may have more severe clinical features than pediatric MCD pts. Several therapeutic approaches have been attempted in nephrotic adult-MCD, but the best treatment, able to assure rapiddly induced, long time remission with few adverse events, is far from being established. Adult-onset MCD patients have been reported to be at higher risk of acute kidney injury with delayed response to glucocorticosteroids as compared to pediatric pts [6]. 30% of adult pts achieve remission over the 8 week course of therapy which is thought to be sufficient in the majority of pediatric cases. Repeated and prolonged steroid therapy is associated to a number of side effects [8]
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