Rossana Malatesta-Muncher1, Karen W Eldin2, Laurence H Beck3, Mini Michael4. 1. Division of Nephrology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA. rxmalate@texaschildrens.org. 2. Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, USA. 3. Division of Nephrology, Department of Medicine, Boston University School of Medicine, Boston, MA, USA. 4. Division of Nephrology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, 1102 Bates Avenue, Suite 245, Houston, TX, 77030, USA.
Abstract
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab. Both patients received two doses of rituximab and responded with remission. In addition, anti-PLA2R antibody levels normalized and/or significantly improved. CONCLUSIONS: Rituximab seems to be a safe and effective treatment option in children with idiopathic membranous nephropathy due to anti-PLA2R. Further studies are needed to evaluate this effectiveness.
BACKGROUND:Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab. Both patients received two doses of rituximab and responded with remission. In addition, anti-PLA2R antibody levels normalized and/or significantly improved. CONCLUSIONS:Rituximab seems to be a safe and effective treatment option in children with idiopathic membranous nephropathy due to anti-PLA2R. Further studies are needed to evaluate this effectiveness.
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