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Effective use of rituximab in combination with low dose cyclophosphamide in childhood onset systemic lupus erythematosus (SLE) with relapsing class IV nephritis

Objectives

We evaluated effectiveness of rituximab, an anti-CD20 monoclonal antibody, in combination with low dose cyclophosphamide and intravenous (IV) methylprednisolone in three pediatric SLE patients with relapsing class IV nephritis.

Methods

Patients 1 and 2

Identical twin females with SLE, complicated by biopsy proven class IV nephritis at age 6-years and treated with NIH cyclophosphamide protocol (NIHCP), had biopsy documented Class IV renal flare at age 10-years; unresponsive to mycophenolate mofetil (MMF) and corticosteroids.

Patient 3

A 12-year old female with SLE, complicated by biopsy documented Class IV nephritis and treated with NIHCP, had 2 further renal flares, which responded to a cumulative dose of 36 grams of cyclophosphamide. At age 16, she had another biopsy documented class IV renal flare, despite maintenance with MMF.

All patients received pulse therapy with: IV cyclophosphamide 0.5 g/m2 with IV methylprednisolone (IVMP) 250 mg on days 1 and 23; IV rituximab 375 mg/m2 on days 2, 9, 16 and 23.

Results

All 3 patients had a dramatic improvement in urine protein-creatinine ratios, and normalization of blood pressure. Despite low-dose daily corticosteroids, all patients had an increase in generalized SLE activity by 4 weeks following rituximab therapy, which responded to re-introduction of MMF. No side effects apart from expected decrease in B-cell counts were noted.

Conclusion

Rituximab in combination with low dose cyclophosphamide and IVMP was effective in controlling recurrent class IV nephritis in 3 pediatric SLE patients.

Re-introduction of mycophenolate mofetil was required within 4 weeks following rituximab therapy to maintain disease remission.

Author information

Correspondence to PM Miettunen.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Systemic Lupus Erythematosus
  • Nephritis
  • Mycophenolate Mofetil
  • Systemic Lupus Erythematosus Activity
  • Renal Flare