Abstract
Background
Immunoglobulin A vasculitis (IgAV), also known as Henoch- Schönlein purpura (HSP), is the most common systemic vasculitis in childhood. Potential differences in demographic characteristics, clinical presentation, laboratory findings, and treatments approaches across age groups, remain poorly explored. To the best of our knowledge, no multicenter study in Latin America has systematically addressed these features. We aimed to assess demographic, clinical and laboratory features, and treatments in children versus adolescents with (IgAV)/ (HSP) in a large multicenter study.
Methods
A multicenter study involving four tertiary centers evaluated 687 children and adolescents (≤ 18 years-old) with IgAV/HSP (EULAR/PRINTO/PRES classification criteria) at first 3 months after diagnosis. The charts were retrospectively assessed for demographic data, initial clinical manifestations, laboratory tests and treatments. Data were compared between children (< 10 years-old) and adolescents (≥ 10 years-old), according to WHO definition.
Results
IgAV/HSP was diagnosed in 599/687(87%) children [5.33(0.88–9.91) years-old] and 88/687(13%) adolescents [11.33(10-17.5) years-old]. The median duration of purpura/petechiae was significantly lower in children compared to adolescents [14(1-120) vs. 15(2–90) days, p = 0.04]. The frequency of persistent purpura/petechiae (≥ 6 weeks of duration) was significantly reduced in the former group (7.2% vs. 19.5%, p = 0.002), likewise the frequency of gastrointestinal bleeding (17% vs. 34.1%, p = 0.01) and proteinuria (49.7% vs. 84%, p = 0.002). In contrast, the frequencies of arthritis/arthralgia (82.7% vs. 73%, p = 0.03) and orchitis(16.6% vs. 4.8%, p = 0.04) were significantly higher in children. Further analysis of laboratory tests showed that the median value of serum IgA was significantly lower in children than in adolescents [179.1(40-1002.0) vs. 279.0(104.0-488.0) mg/dL, p = 0.01], whereas thrombocytosis was higher (40.1% vs. 23%, p = 0.007). Logistic regression demonstrated that persistent purpura/petechiae after IgAV/HSP diagnosis (OR = 18.337; 95%CI 1.245-270.137; p = 0.034) was the only independently associated variable with dependent variable (adolescent).
Conclusions
In this large multicenter cohort, IgAV/HSP onset occurred rarely at adolescence, with a more prominent cutaneous involvement. Prolonged purpura/petechiae after IgAV/HSP diagnosis was associated with adolescent-onset IgAV/HSP, reinforcing the need for vigilant monitoring in this subgroup.
Data availability
All data generated or analyzed during this study are included in this published article [and its supplementary information files].
Abbreviations
- BMI:
-
Body mass index
- CKD:
-
Chronic kidney disease
- CRP:
-
C–reactive protein
- DLCO:
-
Diffusing capacity for carbon monoxide
- EULAR:
-
European League Against Rheumatism
- ESR:
-
Erythrocyte sedimentation rate
- HSP:
-
Henoch–Schönlein purpura
- HRCT:
-
Resolution computed tomography
- IgAV:
-
Immunoglobulin A vasculitis
- IVIG:
-
Immunoglobulin
- KDIGO:
-
Kidney Disease Improving Global Outcomes
- PRES:
-
Pediatric Rheumatology European Society
- PRINTO:
-
Pediatric Rheumatology International Trials Organization
- PRES:
-
Posterior reversible encephalopathy syndrome
- SD:
-
Standard deviation
- WHO:
-
World Health Organization
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Acknowledgements
The authors thank all pediatric specialists at our university hospitals for their follow-up of the IgAV patients in this research.
Funding
This study was supported by grants from Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) (#2022/13837-5 to KK & MC-S) and (#2022/12925-8 to NEA & CAS) and from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) (#304984/2020-5 to CAS).
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This study was conducted in accordance with the principles of the Declaration of Helsinki. The Institutional Ethical Board of HCFMUSP [Comissão de Ética para Análise de Projetos de Pesquisa (CAPPesq)] approved the study (number 70992423.8.1001.0068), as well as the other participating centers.
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For this type of study, formal consent is not required. The need for informed consent was waived by the local ethics committees of the participating centers due to the retrospective nature of the study.
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Communicated by Caio Machado
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Forero, L.F.C., Marra, P.S., Machado, R.N. et al. Prolonged skin involvement distinguishes adolescent from childhood-onset IgA vasculitis: a large multicenter study with 687 patients. Adv Rheumatol (2026). https://doi.org/10.1186/s42358-025-00516-w
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DOI: https://doi.org/10.1186/s42358-025-00516-w