Zespół Schoenleina-Henocha formÄ nefropatii IgA u dzieci?

Schoenlein-Henoch purpura (SHP) is a form of systemic vasculitis characterized by vascular wall deposits of predominantly IgA, typically involving small vessels in skin, gut and glomeruli and associated with purpura, colic pain, haematuria and arthralgia or arthritis. Schoenlein-Henoch nephritis (SHN) leads to chronic renal failure in up to 20% of pediatric patients after 20 years of follow-up in selected series. The risk is related to the initial clinical presentation and the percentage of glomeruli presenting with epithelial crescents. The pathogenesis of SHN might be related to an increased production of abnormally glycosylated IgA, which is not sufficiently cleared by the liver and leads to the formation of IgA macromolecules, accumulating in the circulation with subsequent deposition in vessel walls and in the glomerular mesangium. SHN is related to the IgA nephropathy. No consensus about treatment has been reached up to now. Recent studies indicate that early treatment with methylprednisolone or a combination of steroids and cytotoxic drugs might prevent evolution to chronic renal failure.