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Rheumatology Advance Access published online on December 20, 2005

Rheumatology, doi:10.1093/rheumatology/kei259
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received April 15, 2005
Accepted November 15, 2005

Original Papers

The epidemiology of Wegener's granulomatosis and microscopic polyangiitis in a Southern Hemisphere region

A. Gibson 1, L. K. Stamp 2, P. T. Chapman 2, and J. L. O'Donnell 3 *

1 Department of Rheumatology, Immunology and Allergy, , University of Otago, New Zealand
2 Department of Rheumatology, Immunology and Allergy, , University of Otago, New Zealand; Christchurch School of Medicine and Health Sciences, University of Otago, New Zealand
3 Department of Rheumatology, Immunology and Allergy, , University of Otago, New Zealand; Canterbury Health Laboratories, Canterbury District Health Board (CDHB), New Zealand; Christchurch School of Medicine and Health Sciences, University of Otago, New Zealand

* To whom correspondence should be addressed.
J. L. O'Donnell, E-mail: john.odonnell{at}cdhb.govt.nz


   Abstract

Objective. To determine the prevalence of Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) in the province of Canterbury, New Zealand.

Method. Three hospital clinical databases and the immunology laboratory database were searched and case notes reviewed for patients fulfilling either the 1990 American College of Rheumatology (ACR) criteria for WG or a modification of those criteria that allowed for antineutrophil cytoplasmic antibody (ANCA) positivity in the absence of granulomatous vasculitis. MPA was defined by the Chapel Hill consensus definition; however, in the absence of histological evidence of pauci-immune glomerulonephritis, ANCA positivity in association with evidence of active glomerular disease was included as a criterion. The point prevalence at 31 December 2003 and the 5-yr period prevalence for the interval 1 January 1999 to 31 December 2003 were calculated.

Results. Seventy-three patients with WG and 28 patients with MPA fulfilled the inclusion criteria. A 5-yr period prevalence of 152 WG cases/million [95% confidence interval (CI) 117-186] and 58 MPA cases/million (95% CI 37-80) was calculated using 2001 census data as denominator. Nineteen patients with WG died and 10 patients with MPA died during the study period, resulting in a point prevalence for survivors at 31 December 2003 of 112 cases/million (95% CI 82-142) and 37 cases/million (95% CI 20-55), respectively. Using unmodified ACR criteria the 5-yr period and point prevalence for WG were 131/million (95% CI 99-163) and 93.5/million (95% CI 66-121), respectively. Apart from respiratory tract involvement, which formed part of the case definition of WG, organ involvement was similar in both diseases.

Conclusion. The prevalence of WG and MPA in Canterbury is the highest reported to date. Restricting the case definition of WG to the ACR classification criteria we found a prevalence equivalent to that described in northern Norway. The clinical severity and serological characteristics were similar to descriptions in other WG and MPA patient cohorts. Studies of disease prevalence in other Southern Hemisphere centres will determine if the observed north-south negative disease gradient in the Northern Hemisphere is reciprocated.

Keywords: Wegener's granulomatosis; Microscopic polyangiitis; Epidemiology; Southern Hemisphere..
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