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Epidemiology of primary systemic vasculitis in children : a population-based study from southern Sweden

Mossberg, M. LU ; Segelmark, M. LU ; Kahn, R. LU ; Englund, M. LU and Mohammad, A. LU (2018) In Scandinavian Journal of Rheumatology p.1-8
Abstract

Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch–Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu’s arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1.29 million; 21.4% aged <18 years). Case records for children (0–17 years) assigned a diagnosis code between M300 and M319 and/or D690 were reviewed to ascertain... (More)

Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch–Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu’s arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1.29 million; 21.4% aged <18 years). Case records for children (0–17 years) assigned a diagnosis code between M300 and M319 and/or D690 were reviewed to ascertain diagnosis. Only patients diagnosed between 2004 and 2014 were included. Results: In total, 556 patients with PSV were identified. The annual incidence rate per million children (95% confidence interval) was estimated to be 200 (183–217) for all PSV, 175.5 for IgAV (160–191), 20.1 for KD (14.9–25.4), 1.4 (0–2.8) for each of GPA and MPA, 0.7 (0–1.7) for PAN, and 0.4 (0–1.1) for each of EGPA and TAK. Among children aged <10 years, 99.5% of cases were either IgAV or KD, both exhibiting a seasonal pattern paralleling infections. There were no deaths, but three cases of end-stage renal disease were noted, all in MPA. Conclusions: Vasculitis is relatively common during childhood. Mild cases associated with the infection season are most common in the youngest age groups, while during adolescence a substantial proportion has more severe forms of vasculitis.

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Contribution to journal
publication status
in press
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in
Scandinavian Journal of Rheumatology
pages
8 pages
publisher
Taylor & Francis
external identifiers
  • scopus:85041515000
ISSN
0300-9742
DOI
10.1080/03009742.2017.1412497
language
English
LU publication?
yes
id
8b35e134-4db1-422a-bc2d-9cd5382f0a8a
date added to LUP
2018-03-01 10:45:04
date last changed
2018-07-01 04:52:51
@article{8b35e134-4db1-422a-bc2d-9cd5382f0a8a,
  abstract     = {<p>Objectives: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. Methods: Potential cases of PSV [IgA vasculitis (IgAV, Henoch–Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu’s arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1.29 million; 21.4% aged &lt;18 years). Case records for children (0–17 years) assigned a diagnosis code between M300 and M319 and/or D690 were reviewed to ascertain diagnosis. Only patients diagnosed between 2004 and 2014 were included. Results: In total, 556 patients with PSV were identified. The annual incidence rate per million children (95% confidence interval) was estimated to be 200 (183–217) for all PSV, 175.5 for IgAV (160–191), 20.1 for KD (14.9–25.4), 1.4 (0–2.8) for each of GPA and MPA, 0.7 (0–1.7) for PAN, and 0.4 (0–1.1) for each of EGPA and TAK. Among children aged &lt;10 years, 99.5% of cases were either IgAV or KD, both exhibiting a seasonal pattern paralleling infections. There were no deaths, but three cases of end-stage renal disease were noted, all in MPA. Conclusions: Vasculitis is relatively common during childhood. Mild cases associated with the infection season are most common in the youngest age groups, while during adolescence a substantial proportion has more severe forms of vasculitis.</p>},
  author       = {Mossberg, M. and Segelmark, M. and Kahn, R. and Englund, M. and Mohammad, A.},
  issn         = {0300-9742},
  language     = {eng},
  month        = {02},
  pages        = {1--8},
  publisher    = {Taylor & Francis},
  series       = {Scandinavian Journal of Rheumatology},
  title        = {Epidemiology of primary systemic vasculitis in children : a population-based study from southern Sweden},
  url          = {http://dx.doi.org/10.1080/03009742.2017.1412497},
  year         = {2018},
}