nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-993Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИCASE REPORTSТромботическая микроангиопатия при ювенильном дерматомиозите (Клиническое наблюдение)Thrombotic microangiopathy associated with juvenile dermatomyositisКаганМ. Ю.KaganM. Yu.mkaganorenburg@yahoo.comБервинаН. Н.BervinaN. N.noemail@neicon.ruВоробьеваО. А.VorobyevaO. A.noemail@neicon.ruГУЗ «Областная детская клиническая больница», г. ОренбургRussian FederationГУЗ «Ленинградское областное патолого-анатомическое бюро» г. Санкт-ПетербургRussian Federation201017062025122115118Copyright © Каган М.Ю., Бервина Н.Н., Воробьева О.А., 20252025Каган М.Ю., Бервина Н.Н., Воробьева О.А.Kagan M.Y., Bervina N.N., Vorobyeva O.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/993

Тромботическая микроангиопатия (ТМА) редкое проявление ювенильного дерматомиозита (ЮДМ). Своевременная диагностика этого состояния имеет важное терапевтическое значение, так как дополнительно к иммуносупрессивной терапии дерматомиозита может потребоваться применение инфузий свежезамороженной плазмы, плазмафереза и диализа. Мы приводим наблюдение 4-летнего мальчика с ЮДМ, у которого развилась ТМА. Начальная терапия высокими дозами метилпреднизолона в сочетании с внутривенным введением иммуноглобулина не дала положительного эффекта. Были добавлены инфузии свежезамороженной плазмы и циклофосфан. Отмечался ответ на терапию с достижением полной клинико-лабораторной ремиссии заболевания.

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.

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The authors declare that there are no conflicts of interest present.