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Rheumatology Advance Access published online on October 1, 2009
Rheumatology, doi:10.1093/rheumatology/kep304
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and polyarteritis nodosa

Aladdin J. Mohammad1,2, Lennart T. H. Jacobsson3, Kerstin W. A. Westman4, Gunnar Sturfelt5 and Mårten Segelmark1

1Department of Nephrology, Lund University Hospital, Lund, 2Department of Internal Medicine, Helsingborg Hospital, Helsingborg, 3Department of Rheumatology, 4Department of Nephrology and Transplantation, Malmö University Hospital, Malmö and 5Department of Rheumatology, Lund University Hospital, Lund, Sweden.

Correspondence to: Aladdin J. Mohammad, Department of Nephrology, Lund University Hospital, S-221 85 Lund, Sweden. E-mail: aladdin.mohammad{at}med.lu.se


  Abstract

Objective. To estimate the incidence of and survival rates for WG, microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS) and PAN within a defined population in southern Sweden.

Methods. Cases were retrieved using hospital records and a serology database. All new cases of WG, MPA, CSS and PAN between 1997 and 2006 were included, provided they met pre-defined criteria, and were followed until 30 June 2008. The study area comprised two health care districts with a total population of 641 000. The standardized mortality ratio (SMR) was estimated using Swedish population data as a reference.

Results. A total of 140 (WG, 63; MPA 65; CSS 6; and PAN 6) cases (52% women) with a median age of 67.6 (range 20–96) years fulfilled the inclusion criteria. The annual incidence per million of the population (95% CI) was estimated to be 9.8 (7.4–12.2) for WG, 10.1 (7.7–12.6) for MPA and 0.9 (0–1.7) for both CSS and PAN. The highest incidence was found in patients aged >=75 years (79.1/million). The 1- and 5-year survival rates were 87.8 and 71.6% for all patients, but lower for MPA (80 and 55%) compared with WG (95 and 83%; P = 0.001), although the difference was not significant in the multivariate analysis. The SMR was 2.77 (95% CI 2.02, 3.71) for all patients.

Conclusions. The incidence of WG and MPA was equal in our district, but there was a difference in survival rates related to age and renal function. A progressive increase in age-specific incidence rates was observed.

KEY WORDS: Incidence, Vasculitis, ANCA, Arteritis, Wegener's granulomatosis

Submitted 17 April 2009; revised version accepted 14 August 2009.
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