Arthritis & Rheumatism, Volume 60,
October 2009 Abstract Supplement

The 2009 ACR/ARHP Annual Scientific Meeting
Philadelphia October 16-21, 2009.

Risk of Malignancy in Patients Treated for ANCA-Associated Vasculitides: Follow-up Data From Clinical Trials of the European Vasculitis Study Group (EUVAS)

Heijl1,  Caroline, Harper2,  Lorraine, Flossmann3,  Oliver, Stucker4,  Isabelle, Scott5,  David GI, Watts6,  Richard, Hoglund7,  Peter

University Hospital MAS, Malmö, Sweden
School of Infection and Immunity, Birmingham, United Kingdom
Royal Berkshire Hospital, Reading, United Kingdom
INSERM U754, Villejuif, France
Norfolk and Norwich University Hospital, Norwich, United Kingdom
University of East Anglia, Ipswich, United Kingdom
Lund University Hospital, Lund, Sweden
Hospital Cochin, Paris, France


Standard immunosuppressive treatment for the ANCA-associated vasculitides (AAV), Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA), may pose a significant risk for the development of malignancies. Previous studies suggested that WG treatment was associated with higher incidences of bladder cancer, non-Hodgkin lymphoma (NHL), leukemia and/or non-melanoma skin cancer (NMSC). This study assessed the malignancy risk for patients treated for AAV in the framework of 4 EUVAS trials.


This analysis was based on the 535 patients with newly diagnosed WG or MPA from 14 European countries (and Mexico) who had been enrolled between 1995 and 2002 in the NORAM, CYCAZAREM, CYCLOPS and MEPEX trials. The 4 trials covered the wide spectrum of severities in AAV presentation and evaluated distinct severity-adapted treatment regimens. Over the period 2004–2007, study participants' follow-up events, including cancers diagnosed, were updated by a cross-sectional mailing survey. Age-, sex- and area-standardized incidence ratios (SIR) were calculated by linkage to 5 European cancer registries. Cumulative malignancy rates were computed using the Kaplan–Meier method.


Follow-up data were obtained for 467 (87.3%) patients. The overall cohorts' median time of observation was 5.16 years (range: 0.01–11.46), corresponding to 2648 person-years. During that time, 50 malignancies were diagnosed in 46 patients, including 14 NMSC, 4 bladder cancers, 1 NHL and 2 leukemias. The cumulative 5-year cancer rate was 8.6% (95% CI: 5.7–11.5). SIR were: all-sites malignancies, 1.48 (95% CI: 1.10–1.96); all-sites malignancies excluding NMSC, 1.28 (95% CI: 0.89–1.77); bladder cancer, 2.26 (95% CI: 0.62–5.78); NHL, 1.02 (95% CI: 0.03–5.69); and leukemia, 3.25 (95% CI: 0.39–11.75). Subgroup SIR for all-sites malignancies were 1.79 (95% CI: 1.23–2.53) for WG, 1.14 (95% CI: 0.67–1.80) for MPA, and 1.48 (95% CI: 1.08–1.97) for cyclophosphamide-treated patients.


Cancer rates for AAV patients treated with conventional immunosuppressive therapy exceeded the expected general population numbers. The smaller magnitude of malignancy risk in this patient cohort compared to previous studies might reflect less extensive use of cyclophosphamide in current treatment protocols.

To cite this abstract, please use the following information:
Heijl, Caroline, Harper, Lorraine, Flossmann, Oliver, Stucker, Isabelle, Scott, David GI, Watts, Richard, et al; Risk of Malignancy in Patients Treated for ANCA-Associated Vasculitides: Follow-up Data From Clinical Trials of the European Vasculitis Study Group (EUVAS) [abstract]. Arthritis Rheum 2009;60 Suppl 10 :645
DOI: 10.1002/art.25725

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