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Thrombotic microangiopathy associated with juvenile dermatomyositis

Abstract

Thrombotic microangiopathy is a rare manifestation of juvenile dermatomyositis. Such patients can develop acute kidney injury. Recognition of this manifestation has important therapeutic implications as the patients may require therapy with plasma exchange and dialysis in addition to the treatment of dermatomyositis. Here, we report a case of a 4-year-old boy with juvenile dermatomyositis with thrombotic microangiopathy. The patient initially received pulses of methylprednisolone and intravenous immunoglobulin which did not induce a significant improvement. He was given infusion of fresh frozen plasma and pulse of cyclophosphamide. The patient responded successfully to this therapy and achieved total remission.

About the Authors

M. Yu. Kagan
ГУЗ «Областная детская клиническая больница», г. Оренбург
Russian Federation


N. N. Bervina
ГУЗ «Областная детская клиническая больница», г. Оренбург
Russian Federation


O. A. Vorobyeva
ГУЗ «Ленинградское областное патолого-анатомическое бюро» г. Санкт-Петербург
Russian Federation


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Review

For citations:


Kagan M.Yu., Bervina N.N., Vorobyeva O.A. Thrombotic microangiopathy associated with juvenile dermatomyositis. Nephrology and Dialysis. 2010;12(2):115-118. (In Russ.)

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ISSN 1680-4422 (Print)
ISSN 2618-9801 (Online)