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

Mohammad, Aladdin LU ; Jacobsson, Lennart LU ; Westman, Kerstin LU ; Sturfelt, Gunnar LU and Segelmark, Mårten LU (2009) In Rheumatology (Oxford, England) 48. p.1560-1565
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... (More)
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. (Less)
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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Rheumatology (Oxford, England)
volume
48
pages
1560 - 1565
publisher
Oxford University Press
external identifiers
  • wos:000272081000022
  • pmid:19797309
  • scopus:75649143613
  • pmid:19797309
ISSN
1462-0332
DOI
10.1093/rheumatology/kep304
language
English
LU publication?
yes
id
1fae298b-44fa-4ebc-bca0-ce44cdc340f2 (old id 1500770)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19797309?dopt=Abstract
http://rheumatology.oxfordjournals.org/content/48/12/1560.short
date added to LUP
2016-04-04 09:21:19
date last changed
2022-04-23 20:13:07
@article{1fae298b-44fa-4ebc-bca0-ce44cdc340f2,
  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.}},
  author       = {{Mohammad, Aladdin and Jacobsson, Lennart and Westman, Kerstin and Sturfelt, Gunnar and Segelmark, Mårten}},
  issn         = {{1462-0332}},
  language     = {{eng}},
  pages        = {{1560--1565}},
  publisher    = {{Oxford University Press}},
  series       = {{Rheumatology (Oxford, England)}},
  title        = {{Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa.}},
  url          = {{http://dx.doi.org/10.1093/rheumatology/kep304}},
  doi          = {{10.1093/rheumatology/kep304}},
  volume       = {{48}},
  year         = {{2009}},
}