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Rheumatology Advance Access published online on August 17, 2004

Rheumatology, doi:10.1093/rheumatology/keh374
Rheumatology © British Society for Rheumatology 2004; all rights reserved
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Received February 18, 2004
Accepted July 13, 2004

Original Papers

Malignancy is increased in ANCA-associated vasculitis

T. Pankhurst 1, C. O. S. Savage 1, C. Gordon 2, L. Harper 1*

1 Division of Medical Sciences, The Medical School, University of Birmingham, Birmingham, UK
2 Division of Immunology and Infection, The Medical School, University of Birmingham, Birmingham, UK

* To whom correspondence should be addressed. E-mail: l.harper{at}bham.ac.uk.


   Abstract

Objective. In the light of previous reports of an association between malignancy and renal vasculitis, we aimed to investigate the association of malignancy in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, either Wegener's granulomatosis (WG) or microscopic polyangiitis (MPA), and compare it with the general population and disease control groups comprising patients with systemic lupus erythematosus (SLE) or Henoch-Schönlein purpura (HSP).

Methods. A retrospective review of 200 consecutive patients with WG or MPA was performed. Malignancies preceding or concurrent with vasculitis were recorded and the incidence of malignancy was compared with those in a population of 129 patients with HSP, 333 patients with SLE and a normal population in the West Midlands of the UK.

Results. Twenty patients had a diagnosis of malignancy, 14 had MPA and six had WG. Patients with ANCA-associated vasculitis had an increased risk of malignancy compared with HSP patients, of whom six patients had malignancy (relative risk 0.85, confidence interval 0.69-1.05; P = 0.034), or SLE patients, of whom five patients had malignancy (relative risk 0.31, 95% confidence interval 0.14-0.7; P<0.0001). The rate of malignancy compared with an age-matched control group was increased in patients with ANCA-associated vasculitis and HSP (ANCA-associated vasculitis, relative risk 6.02, 95% confidence interval 3.72-9.74; HSP, relative risk 5.25, 95% confidence interval 2.4-11.5). The presence of ANCA was not predictive of malignancy.

Conclusion. In conclusion, patients with ANCA-associated vasculitis have an increased risk of preceding or concurrent malignancy.

Keywords: Vasculitis; Renal; Malignancy; Cancer; Wegener's granulomatosis; Henoch-Schönlein purpura.
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