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Научно-практическая ревматология

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Синдром активации макрофагов у больных системным ювенильным артритом

https://doi.org/10.14412/1995-4484-2014-202-208

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Аннотация

Синдром активации макрофагов (САМ) является одним из гистиоцитарных заболеваний, развивающихся из клеток макрофагального ряда, – гемофагоцитарным лимфогистиоцитозом (ГЛГ). Показано, что ревматиче- ские заболевания нередко сопровождаются развитием CАМ, наиболее часто – при системном варианте юве- нильного артрита (сЮА). Рассматриваются отдельные вопросы патогенеза с концепцией дефекта механиз- мов Т-клеточной цитотоксичности и снижения уровня активности естественных киллеров (NK), сопряжен- ных с мутацией в гене, кодирующем перфорин, – PRF1, а также гиперпродукции Т-лимфоцитами и гистио- цитами ряда цитокинов (интерлейкина 1β – ИЛ1β, интерферона γ, фактора некроза опухоли α, растворимо- го ИЛ2-рецептора), опосредованно ведущих к активации тканевых макрофагов и продукции провоспали- тельных цитокинов. Обсуждаются проблемы диагностики, связанные с низкой чувствительностью и специ- фичностью диагностических критериев гемофагоцитарного синдрома HLH 2010 г., опирающихся на молеку- лярную генетику и патоморфологическую диагностику ГЛГ. Представлены диагностические критерии син- дрома активации макрофагов (2012), разработанные для сЮА, с ведущим значением тромбоцитопении, ги- перферритинемии и патогистологических признаков гемофагоцитоза. Обращено внимание на необходи- мость диагностики субклинических и «мягких» форм САМ, выявления потенциальной группы риска и пре- дотвращения развития САМ. Рассмотрены вопросы дифференциальной диагностики, учитывая схожесть клинических проявлений с сЮА, терапевтической тактики с применением протокола HLH 2004 г., биологи- ческой терапии. 

Об авторах

С. Р. Родионовская
ФГБУ «Научно- исследовательский институт им. В.А. Насоновой» РАМН, Москва, Россия
Россия


И. П. Никишина
ФГБУ «Научно- исследовательский институт им. В.А. Насоновой» РАМН, Москва, Россия
Россия


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Для цитирования:


Родионовская С.Р., Никишина И.П. Синдром активации макрофагов у больных системным ювенильным артритом. Научно-практическая ревматология. 2014;52(2):202-208. https://doi.org/10.14412/1995-4484-2014-202-208

For citation:


Rodionovskaya S.R., Nikishina I.P. MACROPHAGE ACTIVATION SYNDROME IN PATIENTS WITH SYSTEMIC JUVENILE ARTHRITIS. Rheumatology Science and Practice. 2014;52(2):202-208. (In Russ.) https://doi.org/10.14412/1995-4484-2014-202-208

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ISSN 1995-4484 (Print)
ISSN 1995-4492 (Online)