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Extended report
Rituximab for refractory granulomatosis with polyangiitis (Wegener's granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations
  1. Julia U Holle1,
  2. Christin Dubrau1,
  3. Karen Herlyn1,
  4. Martin Heller2,
  5. Petra Ambrosch3,
  6. Bernhard Noelle4,
  7. Eva Reinhold-Keller1,
  8. Wolfgang L Gross1
  1. 1Department of Rheumatology and Clinical Immunology, Vasculitis Center, University Hospital Schleswig-Holstein, Campus Lübeck and Klinikum Bad Bramstedt, Bad Bramstedt, Germany
  2. 2Department of Diagnostic Radiology, University of Kiel, Kiel, Germany
  3. 3Department of Otorhinolaryngology, University of Kiel, Kiel, Germany
  4. 4Department of Ophthalmology, University of Kiel, Kiel, Germany
  1. Correspondence to Dr Julia U Holle, Department of Rheumatology and Clinical Immunology, Vasculitis Center, University Hospital Schleswig-Holstein/Klinikum Bad Bramstedt, Oskar-Alexander-Strasse 26, 24576 Bad Bramstedt, Germany; hollejulia{at}


Objective First, to investigate the overall efficacy and safety of rituximab (RTX) in refractory granulomatosis with polyangiitis (GPA) in a tertiary referral centre. Second, to compare the efficacy of RTX in granulomatous and vasculitic manifestations in GPA.

Patients and methods This study comprised a retrospective, standardised data collection from all patients who received RTX for refractory Wegener's granulomatosis from 2002 to 2010. Patients were assessed by a standardised interdisciplinary diagnostic procedure (including ear, nose and throat and ophthalmology assessment, MRI, immunodiagnostics, B-cell levels and Birmingham Vasculitis Activity Score) and were treated by standardised therapeutic regimens according to available evidence.

Results 59 patients received 75 cycles of RTX. 9.3% achieved complete remission. A response was documented in 61.3% (improvement in 52%, unchanged disease activity in 9.3%), 26.7% had refractory disease. Birmingham Vasculitis Activity Score, disease extent index, erythrocyte sedimentation rate, C-reactive protein and prednisolone demand decreased significantly. All patients achieved B-cell depletion. Granulomatous manifestations such as orbital granuloma and pachymeningitis were more frequently refractory to RTX than vasculitis or other granulomatous manifestations. Thus, for example, complete remission/improvement was found in 89.2% of patients with renal disease and in only 44.4% of those with orbital masses (p=0.003). The relapse rate was 44.4% after a median period of 13.5 months. Adverse events occurred in 29%, pneumonia in 15% and death in 3%.

Conclusion The overall response rate of refractory GPA to RTX was high (61.3% complete remission or improvement). Response rates of vasculitic manifestations were excellent; failure of response/progress was mostly due to granulomatous manifestations, especially orbital masses. Relapse rates were high (40%) despite maintenance treatment.

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  • Competing interests None.

  • Ethics approval Ethics approval was obtained from University of Luebeck.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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