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Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials
  1. C Heijl1,
  2. L Harper2,
  3. O Flossmann3,
  4. I Stücker4,
  5. DGI Scott5,
  6. R A Watts6,
  7. P Höglund7,
  8. K Westman8,
  9. A Mahr9,
  10. for the European Vasculitis Study Group (EUVAS)
  1. 1Department of Nephrology, Skåne University Hospital Lund, Lund University, Lund, Sweden
  2. 2Department of Nephrology, School of Infection and Immunity, University of Birmingham, Birmingham, UK
  3. 3Department of Nephrology, Royal Berkshire Hospital Reading, Reading, UK
  4. 4INSERM U754, Villejuif, France
  5. 5Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
  6. 6School of Medicine, Health Policy and Practice, University of East Anglia, Ipswich, UK
  7. 7Department of Clinical Pharmacology, Department of Laboratory Medicine, Skåne University Hospital Lund, Lund University, Lund, Sweden
  8. 8Department of Nephrology and Transplantation, Skåne University Hospital Malmö, Lund University, Malmö, Sweden
  9. 9Department of Internal Medicine, Hospital Cochin, Paris, France
  1. Correspondence to Dr A Mahr, Department of Internal Medicine, Hospital Saint-Louis, 1 avenue Claude-Vellefaux, 75475 Paris Cedex 10, France; alfred.mahr{at}


Objectives Because standard immunosuppressive treatment for antineutrophil cytoplasm antibody-associated vasculitis (AAV) (granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA)) has been associated with a significant risk of developing cancer, the cancer incidence of treated AAV patients was assessed.

Methods This analysis concerned 535 patients with newly diagnosed AAV from 15 countries who had been enrolled between 1995 and 2002 in four European clinical trials. Over the period 2004–7, study participants' follow-up events were updated, including cancers diagnosed. Age, sex and area-standardised incidence ratios (SIR) and their 95% CI were calculated by linkage to five national cancer databases.

Results During the 2650 person-years' observation period, 50 cancers were diagnosed in 46 patients. SIR (95% CI) were 1.58 (1.17 to 2.08) for cancers at all sites, 1.30 (0.90 to 1.80) for cancers at all sites excluding non-melanoma skin cancer (NMSC), 2.41 (0.66 to 6.17) for bladder cancer, 3.23 (0.39 to 11.65) for leukaemia, 1.11 (0.03 to 6.19) for lymphoma and 2.78 (1.56 to 4.59) for NMSC. Subgroup SIR for cancers at all sites were 1.92 (1.31 to 2.71) for GPA and 1.20 (0.71 to 1.89) for MPA.

Conclusions Cancer rates for AAV patients treated with conventional immunosuppressive therapy exceeded those expected for the general population. This cancer excess was largely driven by an increased incidence of NMSC. The smaller cancer risk magnitude in this cohort, compared with previous studies, might reflect less extensive use of cyclophosphamide in current treatment protocols. Longer follow-up data are warranted to appraise the risk of developing cancers later during the course of AAV.

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  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the local ethic committees.

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