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

Scand J Rheumatol. 2018 Jul;47(4):295-302. doi: 10.1080/03009742.2017.1412497. Epub 2018 Feb 7.


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.

MeSH terms

  • Adolescent
  • Age Distribution
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / epidemiology*
  • Child
  • Child, Preschool
  • Churg-Strauss Syndrome / epidemiology
  • Female
  • Granulomatosis with Polyangiitis / epidemiology
  • Humans
  • IgA Vasculitis / epidemiology
  • Incidence
  • Infant
  • Infant, Newborn
  • Kidney Failure, Chronic / epidemiology
  • Male
  • Microscopic Polyangiitis / epidemiology
  • Mucocutaneous Lymph Node Syndrome / epidemiology*
  • Polyarteritis Nodosa / epidemiology*
  • Registries*
  • Seasons*
  • Sex Distribution
  • Sweden / epidemiology
  • Systemic Vasculitis / epidemiology
  • Takayasu Arteritis / epidemiology*