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Rituximab for treatment-resistant extensive Wegener`s granulomatosis—additive effects of a maintenance treatment with leflunomide

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Abstract

Extensive Wegener’s granulomatosis (WG) is treated by glucocorticosteroids (GC) and cyclophosphamide (CYC). In some cases, the disease is refractory to CYC. For those patients the depletion of B-lymphocytes with rituximab is a promising new treatment modality. This is a retrospective study of six patients receiving rituximab (RTX) with 4 × 375 mg/m2 body surface weekly because of inefficacy of CYC. Proteinase-3-antineutrophil cytoplasmic antibodies (PR3-ANCA) and c-ANCAs were assessed. For clinical follow-up the Birmingham Vasculitis Activity Score for WG (BVAS/WG) was used. In five of the six cases, leflunomide (LEF) was given as maintenance treatment. Mean follow up was 16 months (12–21 months). The median PR3-ANCA titer fell from 36.8 U/ml at baseline to 21.4 U/ml after 3 months, 8.3 after 6 months, and 4.3 at month 12. The median BVAS/WG at baseline was 5 and 0 after 1 month. Two minor relapses could be noticed at month 3. After 6 months, one patient still had a BVAS of 1, all the others had a BVAS of 0. At month 18, a major relapse occurred in one patient, which was successfully retreated with RTX. The RTX infusions were well tolerated. Rituximab is a well-tolerated, very effective medication for patients with Wegener’s granulomatosis. Leflunomide maintenance may increase the efficacy of rituximab and prolong the disease-free period.

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Authors and Affiliations

  1. Department of Internal Medicine II (Hematology, Oncology, Immunology, Rheumatology, Pulmology), University Hospital, Otfried-Mueller-Str. 10, 72076, Tuebingen, Germany

    J. C. Henes, S. Koch, R. Klein, L. Kanz & I. Koetter

  2. Department of Radiology, University Hospital, Otfried-Mueller-Str. 10, 72076, Tuebingen, Germany

    J. Fritz & M. Horger

  3. Department of Internal Medicine, Division of Nephrology, University Hospital, Otfried-Mueller-Str. 10, 72076, Tuebingen, Germany

    T. Risler

Authors
  1. J. C. Henes
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  2. J. Fritz
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  3. S. Koch
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  4. R. Klein
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  5. M. Horger
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  6. T. Risler
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  7. L. Kanz
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  8. I. Koetter
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Correspondence to I. Koetter.

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Henes, J.C., Fritz, J., Koch, S. et al. Rituximab for treatment-resistant extensive Wegener`s granulomatosis—additive effects of a maintenance treatment with leflunomide. Clin Rheumatol 26, 1711–1715 (2007). https://doi.org/10.1007/s10067-007-0643-9

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  • DOI: https://doi.org/10.1007/s10067-007-0643-9

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