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Chapter 3.5 - Immunoglobulin A Vasculitis (Henoch–Schönlein Purpura)

from 3 - Hypercoagulable Causes of Stroke

Published online by Cambridge University Press:  06 October 2022

Anita Arsovska
Affiliation:
University of Ss Cyril and Methodius
Derya Uluduz
Affiliation:
Istanbul Üniversitesi
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Summary

Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura is a nonthrombocytopenic, immune-mediated, leukocytoclastic, small vessel vasculitis. It is the most common form of systemic vasculitis in children. Adults are less frequently affected. Most studies show a male predominance. There is a seasonal pattern with rarest occurrence in summer, probably because of the association of IgA vasculitis with infections. Pathophysiologically, immune complex IgA deposition in vessel walls is leading to complement activation, which is causing vessel injury. Neurological manifestation is more frequent in other forms of vasculitis. But, neurological manifestation of IgA vasculitis is possible, but rare. They involve affection of the central and peripheral nervous system. Sokol et al. reported the case of a 15-year-old girl presenting with aphasia and right-sided weakness associated with lethargy. Diagnostic tests led to the diagnosis of IgA vasculitis with neurologic involvement

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Chapter
Information
Rare Causes of Stroke
A Handbook
, pp. 174 - 178
Publisher: Cambridge University Press
Print publication year: 2022

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References

Sokol, DK, McIntre, JA, Short, RA, et al. Henoch-Schonlein purpura and stroke: Antiphosphatidylethanolamine antibody in CSF and serum. Neurology. 2000;55: 1379.CrossRefGoogle ScholarPubMed
Garzoni, L, Vanoni, F, Rizzi, M, et al. Nervous system dysfunction in Henoch-Schonlein syndrome: Systematic review of the literature. Rheumatology. 2009;48: 524529.CrossRefGoogle ScholarPubMed
Oni, L, Sampath, S. Childhood IgA vasculitis (Henoch Schonlein Purpura): Advances and knowledge gaps. Front Pediatr. 2019;27(7): 257.CrossRefGoogle Scholar
González-Gay, MA, López-Mejías, R, Pina, T, et al. IgA vasculitis: Genetics and clinical and therapeutic management. Curr Rheumatol Rep. 2018;20: 24.Google Scholar

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