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

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