Abstract
Henoch-Schonlein purpura (HSP), recently re-named IgA vasculitis, is a systemic small vessel vasculitis with immunoglobulin (Ig) A1-dominant immune deposits. The disease is characterized by a tetrad of clinical manifestations: palpable purpuric rash, arthralgia/arthritis, gastrointestinal symptoms (i.e., abdominal pain, gastrointestinal bleeding), but any organ may be involved. It is considered a form of immune complex-mediated vasculitis, but the etiopathogenesis is not yet completely understood.
The goals of treating HSP are typically to ameliorate acute symptoms, mitigate short-term morbidity and prevent chronic renal insufficiency. Corticosteroids and immunosuppressive agents have been postulated to be effective, but their role remains controversial. The efficacy of Rituximab in adult with IgA vasculitis has been reported in few cases.
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Fenoglio, R., Roccatello, D. (2016). IgA Vasculitis. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_18
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DOI: https://doi.org/10.1007/978-3-319-40136-2_18
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