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Pediatric Rheumatology

, 9:21

First Online: 11 August 2011Received: 07 March 2011Accepted: 11 August 2011


Henoch-Schönlein purpura is one of the most common forms of systemic vasculitis of childhood. We report the response to anakinra, the interleukin-1 receptor antagonist, in a 9 year old girl without prior medical problems who developed life-threatening Henoch-Schönlein vasculitis that produced renal failure, pulmonary hemorrhage and vasculitis of the brain. Her response supports the theory that interleukin-1 may be an important mediator in this disease. Further study of interleukin-1 antagonists in severe Henoch-Schönlein purpura may be warranted.

List of abbreviationsDNAdeoxyribonucleic acid

FMFfamilial Mediterranean fever

HSPHenoch-Schönlein purpura

IgAimmunoglobulin A


IL-1RAinterleukin-1 receptor antagonist

IVIGintravenous immunoglobulin

MEFVgene encoding pyrin, mutated in familial Mediterranean fever

MRmagnetic resonance

MRImagnetic resonance imaging.

Electronic supplementary materialThe online version of this article doi:10.1186-1546-0096-9-21 contains supplementary material, which is available to authorized users.

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Author: Erynn M Boyer - Martin Turman - Kathleen M O-Neil


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