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Published on: June 15, 2020
Pediatric vasculitis
Kenan Barut1, Sezgin Sahin, Ozgur Kasapcopur
1Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
Insights
Childhood vasculitis diagnosis is challenging due to varied symptoms. Recent studies offer new insights into genetic factors, diagnostic tools, and successful biological therapies for pediatric vasculitis.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Vascular Medicine
Background:
- Childhood vasculitis presents with diverse signs and symptoms, complicating diagnosis.
- Recent advancements include new classification criteria and a deeper understanding of genetic predispositions.
- Radiological techniques are increasingly vital for accurate diagnosis.
Purpose of the Study:
- To define childhood vasculitis.
- To identify novel causative factors.
- To review updated treatment strategies based on recent research.
Main Methods:
- Literature review of recently published studies on pediatric vasculitis.
- Analysis of diagnostic criteria, genetic associations, and therapeutic interventions.
- Evaluation of clinical presentations and disease severity in children.
Main Results:
- Henoch-Schonlein purpura and Kawasaki disease are the most common forms in children.
- Antineutrophilic cytoplasmic antibody-associated vasculitis is rare in pediatric populations.
- Biological therapies show promise, mirroring adult treatment successes, with increased reports of severe manifestations like renal disease and subglottic stenosis.
Conclusions:
- Multicenter studies are essential for pediatric vasculitis to establish evidence-based treatment guidelines and prognosis.
- Current treatment and prognostic data often extrapolate from adult studies due to the rarity of other vasculitides in children.
- Further research is needed to develop specific pediatric indices for disease activity and damage.
Purpose Of Review:
The aim of this review is to define childhood vasculitis and to highlight new causative factors and treatment modalities under the guidance of recently published studies.
Recent Findings:
Childhood vasculitis is difficult to diagnose because of the wide variation in the symptoms and signs. New nomenclature and classification criteria were proposed for the diagnosis of pediatric vasculitis. Recently, progress has been made toward understanding the genetic susceptibility to pediatric vasculitis as it was in other diseases. Various radiological techniques provide great opportunities in establishing the diagnosis of pediatric vasculitis. Mild central nervous system disease can accompany Henoch-Schonlein purpura and can go unnoticed. Antineutrophilic cytoplasmic antibody-associated vasculitis is rare in children. Increased severity of the disease, subglottic stenosis, and renal disease are described more frequently among children. Biological therapies are used with success in children as in adults. Future studies, whose aims are to evaluate treatment responses, prognosis and to design guidelines for activity, and damage index of vasculitis for children are required.
Summary:
Henoch-Schonlein purpura and Kawasaki disease are the most frequent vasculitides of children. Experience from adult studies for treatment and prognosis are usually used because of low incidence of other vasculitides in children. Multicenter studies of pediatric vasculitis should be conducted to detail treatment responses and prognosis in children.
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