Rituximab efficacy on complicated refractory nephrotic syndrome: case report

This is a 16-year-old girl presentation who involved nephrotic syndrome (NS) since 11 years ago with biopsy exhibited minimal changed NS. Her full blunt nephrotic presentation was accompanied with normal GFR, no hematuria and without skin rash or hypertension at onset. Her serum complement assays were within normal range and immunofluorescence renal histology was negative. Although initial treatment with oral prednisolone was effective, but complicated by refractory and relapsing course and developed adverse drug toxicity such as cushingoid appearance, hypertension, short stature and low bone density. She received multiple adjuvant therapy with levamisole, Azathioprine, cyclophosphamide, cyclosporine and mycophenolate mofetil. Although Tacrolimus have had dramatic response, finally proteinuria reappear and sever skin rash proven biopsy pustular psoriasis developed. Rituximab (ZytuxTMAryogen) was started instead of tacrolimus, 4 weekly doses followed by one booster at sixth month. Her proteinuria diminished since third week of treatment and she experienced no relapse at one year follow-up with resolved skin rash. These observation suggest the efficacy of rituximab in patients with difficult nephrotic syndromewho had either responded unsatisfactorily to treatment with multiple immunosuppressive agents or had features indicating of medication-related toxicity. Persistent proteinuria known as poor prognostic predictor regarding renal function in nephrotic syndrome and the use of effective and safe medication such as rituximab in patients with refractory and complicated nephrotic syndrome is highly recommended.

Keywords:

nephrotic syndrome , steroid resistance , rituximab , children