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Published on: June 15, 2020
Demographic and clinical features of pediatric vasculitis: a single-center study
Tiago Santos Trindade1, Catarina Granjo Morais2, Ana Maia2
1Faculty of Medicine of the University of Porto, Centro Hospitalar e Universitário de São João.
Insights
This study analyzed pediatric systemic vasculitis cases, finding Kawasaki disease (KD) in younger children and Behçet's syndrome (BS) in adolescents. Most patients with IgA Vasculitis (IgAV) and KD showed high treatment response rates.
Area of Science:
- Pediatric Rheumatology
- Systemic Vasculitis Research
- Childhood Autoimmune Diseases
Background:
- Vasculitides are rare systemic inflammatory conditions affecting children.
- Understanding demographic and clinical features is crucial for diagnosis and management.
- International comparisons highlight variations in presentation and outcomes.
Purpose of the Study:
- To document demographic and clinical features of pediatric systemic vasculitis.
- To compare findings with international studies.
- To identify key differences and similarities in disease presentation.
Main Methods:
- Retrospective analysis of 138 pediatric patients diagnosed between 2009-2020.
- Inclusion criteria: patients under 18 years at diagnosis or follow-up.
- Data collected on demographics, clinical signs, and treatment response.
Main Results:
- Kawasaki disease (KD) presented in younger children (median age 2.26 years) vs. Behçet's syndrome (BS) in adolescents (median age 13.41 years).
- High treatment response (>90%) observed in IgA Vasculitis (IgAV) and KD.
- Gastrointestinal and joint symptoms were common; renal and ophthalmic involvement varied by vasculitis type.
Conclusions:
- Pediatric vasculitides exhibit distinct age distributions and clinical manifestations.
- Findings largely align with literature, with some atypical presentations noted.
- Further multicenter studies are needed to advance research and clinical trials.
Introduction:
Vasculitides are rare systemic conditions which may occur in childhood. This study aims to document demographic and clinical features of systemic vasculitides in a tertiary center, comparing our outcomes with previously published studies of other international centers.
Methods:
Patients presenting with systemic vasculitis before 18 years of age, admitted to a tertiary Portuguese center at diagnosis or during follow-up, from 2009 to 2020, were retrospectively included in this study.
Results:
In our study, we included 138 patients. The youngest patients at diagnosis were in the Kawasaki's disease (KD) group, with a median age at diagnosis of 2.26 years old (IQR 1.07-6.20), as opposed to the Behçet's syndrome (BS) group with a median age at diagnosis of 13.41 years old (IQR 10.19-16.75), which was significantly higher (p 90% in both IgA Vasculitis (IgAV) and KD. Gastrointestinal symptoms were common in all groups (15-50%), rarer in BS (17%). Arthritis and arthralgia were highly prevalent in IgAV (65%). The American Heart Association criteria of diagnosis for complete KD were met in 62% of cases. No significant difference was found in age distribution between complete and incomplete diagnosis (p=0.616). Mean duration of fever in KD was 9.6 ±2.1 days, which was higher than anticipated. Renal manifestations in IgAV (11%) and ophthalmic involvement in BS (22%) were lower than expected. There was a notable number of children reporting joint involvement in KD (27%). We also noticed a slightly higher prevalence of vascular events in BS (30%).
Conclusions:
Each specific vasculitis assessed had different key symptoms, but there are several complaints and signs shown by our patients, some of them overlapping between vasculitides and others very atypical, such as recurrent epididymitis in BS due to microvasculitis. We can state that most of our findings are in concordance with current literature, with some notable exceptions. Pediatric multicentric population-based studies are warranted to increase research and design clinical trials concerning this field of knowledge.
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