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Epidemiology of primary systemic vasculitis in children: a population-based study from southern Sweden
M Mossberg1, M Segelmark2, R Kahn1
1a Department of Paediatrics, Clinical Sciences Lund , Lund University , Lund , Sweden.
Insights
Pediatric primary systemic vasculitis (PSV) affects 200 per million children annually in Sweden. IgA vasculitis and Kawasaki disease are most common in younger children, while adolescents may develop severe forms.
Area of Science:
- Pediatric Rheumatology
- Epidemiology
- Immunology
Background:
- Primary systemic vasculitis (PSV) encompasses a group of rare but serious autoimmune diseases affecting children.
- Understanding the incidence and specific types of PSV in pediatric populations is crucial for public health and clinical management.
Purpose of the Study:
- To determine the annual incidence rate of pediatric primary systemic vasculitis (PSV) in a defined geographical region of southern Sweden.
- To identify the most prevalent types of PSV in children and analyze their age-specific patterns.
Main Methods:
- A comprehensive regional healthcare register in Skåne, Sweden, was utilized to identify potential cases of PSV.
- Case records of children (0-17 years) diagnosed with vasculitis codes (M300-M319 and/or D690) between 2004 and 2014 were reviewed.
- Incidence rates were calculated per million children, with specific rates for IgA vasculitis (IgAV), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK).
Main Results:
- A total of 556 PSV cases were identified, yielding an overall annual incidence of 200 per million children.
- IgA vasculitis (IgAV) was the most common type (175.5/million), followed by Kawasaki disease (KD) (20.1/million).
- Rare forms like GPA, MPA, PAN, EGPA, and TAK had significantly lower incidence rates. IgAV and KD showed seasonal patterns, particularly in children under 10. Three cases of end-stage renal disease were noted, all in MPA patients.
Conclusions:
- Pediatric primary systemic vasculitis is relatively common, with IgAV and KD being the most frequent diagnoses in younger children.
- Seasonal variations in milder vasculitis forms are linked to infections in younger age groups.
- Adolescents are more prone to developing severe forms of vasculitis, highlighting the need for age-specific monitoring and treatment strategies.
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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