Introduction: Although rituximab (Rit) is a greatly effective salvage therapy for refractory nephrotic syndrome (NS), the duration of the efficacy is limited in many cases after the cessation of Rit. We experienced 2 cases of refractory NS successfully treated with re―starting the combination therapy of cyclosporin A (CyA) and mizoribine (MZR) after Rit treatment.
Case 1: The patient is a 10―year―old boy with MCNS, who was first diagnosed at the age of 2 in 2003. Since then, he has experienced 16 relapses even under the combination therapy of CyA and MZR. In 2007, 4 series of Rit administrations were performed at the age of 7. Five months after the last Rit administration, he began to suffer from relapses again. After the combination therapy of CyA and MZR was restarted, he has been free from relapses for 1 year.
Case 2: The patient is a 19―year―old boy with MCNS, who was first diagnosed at the age of 6 in 1997, and underwent 43 relapses under various treatments including the combination therapy of CyA and MZR. In 2009, 4 series of Rit administrations were carried out at the age of 18. He showed another relapse again 8 months after the last Rit administration, and the combination therapy was started again. Since then, no relapse has occurred to date.
Discussion: After Rit treatment, there is a possibility that the effectiveness of the combination therapy of CyA and MZR may change for the better, although this mechanism is not proven. The long―term and recurrent use of CyA certainly increases a risk of CyA nephrotoxicity and repeated renal biopsy might be necessary to detect irreversible drug―induced adverse effects. MZR is also reported to have a function of reducing CyA nephrotoxicity.
Conclusion: Re―starting the combination therapy of CyA and MZR might be one of the choices for the patients with refractory NS after Rit treatment failure.
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