mrjСовременная ревматологияModern Rheumatology Journal1996-70122310-158XIMA-PRESS, LLC10.14412/1996-7012-2015-1-55-59mrj-597Research ArticleРЕВМАТИЧЕСКИЕ ЗАБОЛЕВАНИЯ ДЕТСКОГО ВОЗРАСТАСовременные подходы к диагностике и лечению синдрома активации макрофагов у детей с ревматическими заболеваниямиCurrent approaches to diagnosing and treating macrophage activation syndrome in children with rheumatic diseasesКостикМихаил МихайловичKostikM. M.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

kost-mikhail@yandex.ruДубкоМ. Ф.DubkoM. F.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

МасаловаВ. В.MasalovaV. V.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

СнегиреваЛ. С.SnegirevaL. S.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

ЧиковаИ. А.ChikovaI. A.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

ИсуповаЕ. А.IsupovaE. A.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

КалашниковаО. В.KalashnikovaO. V.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

ЧасныкВ. Г.ChasnykV. G.

194100, Санкт-Петербург, Литовская ул., 2

2, Litovskaya St., Saint Petersburg 194100

ГБОУ ВПО «Санкт-Петербургский государственный педиатрический медицинский университет»РоссияSaint Petersburg State Pediatric Medical UniversityRussian Federation201523032015915559Copyright © Костик М.М., Дубко М.Ф., Масалова В.В., Снегирева Л.С., Чикова И.А., Исупова Е.А., Калашникова О.В., Часнык В.Г., 20152015Костик М.М., Дубко М.Ф., Масалова В.В., Снегирева Л.С., Чикова И.А., Исупова Е.А., Калашникова О.В., Часнык В.Г.Kostik M.M., Dubko M.F., Masalova V.V., Snegireva L.S., Chikova I.A., Isupova E.A., Kalashnikova O.V., Chasnyk V.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://mrj.ima-press.net/mrj/article/view/597

Синдром активации макрофагалов (САМ) – тяжелое, жизнеугрожаемое состояние, проявляющееся гемофагоцитозом, панцитопенией, коагулопатией, нарушением функции печени и ЦНС. Заболевание относится к группе гистиоцитарных расстройств. Основными триггерными факторами САМ являются ревматические заболевания, особенно системная форма ювенильного идиопатического артрита, инфекционные заболевания, иммунодефицит, лекарственные препараты. Описаны основные этапы патогенеза САМ, роль гиперцитокинемии. Представлена клиническая картина САМ и гемофагоцитарных лимфогистиоцитозов. Рассмотрена эволюция диагностических подходов при САМ и родственных состояниях, таких как наследственный и вторичный гемофагоцитарный лимфогистиоцитоз. Показано значение разработки диагностических критериев САМ у пациентов с системной формой ювенильного идиопатического артрита. Изложены современные подходы к терапии САМ.

Macrophage activation syndrome (MAS) is a severe life-threatening complication presenting with hemophagocytosis, pancytopenia, coagulopathy, and liver and CNS dysfunctions. The disease belongs to a group of histiocytic disorders. The common triggers for MAS are rheumatic diseases, particularly systemic juvenile idiopathic arthritis (SJIA), infectious diseases, immunodeficiency, and medication. The paper describes the main stages of the pathogenesis of MAS and the role of hypercytokinemia. It presents the clinical picture of MAS and hemophagocytic lymphohistiocytoses. The evolution of diagnostic approaches to diagnosing MAS and related conditions, such as hereditary and secondary hemophagocytic lymphohistiocytosis, is considered. It is shown that it is important to elaborate diagnostic criteria for patients with SJIA. Main current approaches to therapy for MAS are outlined.

синдром активации макрофаговсемейный гемофагоцитарный лимфогистиоцитозреактивный гемофагоцитарный лимфогистиоцитозсистемная форма ювенильного идиопатического артритаферритининтерлейкин 1macrophage activation syndromefamilial hemophagocytic lymphohistiocytosisreactive hemophagocytic lymphohistiocytosissystemic juvenile idiopathic arthritisferritininterleukin 1ReferencesPrieur AM, Stephan JL. Macrophage activation syndrome in rheumatic diseases in children. Rev Rhum Ed Fr. 1994 Jun;61(6):447–51.Prieur AM, Stephan JL. Macrophage activation syndrome in rheumatic diseases in children. Rev Rhum Ed Fr. 1994 Jun;61(6):447–51.Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 2012 Jun;13(4):289–98. doi: 10.1038/gene.2012.3. Epub 2012 Mar 15.Ravelli A, Grom AA, Behrens EM, Cron RQ. 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The authors declare that there are no conflicts of interest present.