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Вопросы гематологии/онкологии и иммунопатологии в педиатрии

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Научно-практический журнал Международной организации Consensus in Pediatrics Лечение васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами

https://doi.org/10.24287/1726-1708-2016-15-2-5-13

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Аннотация

Васкулиты, ассоциированные с антинейтрофильными цитоплазматическими антителами (АНЦА) (далее ААВ), включают гранулематоз с полиангиитом (ранее болезнь Вегенера, теперь ГП), микроскопический полиангиит (МП), эозинофильный гранулематоз с полиангиитом (ЭГП, ранее - синдром Чердж-Стросса) и заболевания отдельных органов, такие как почечный васкулит. Хотя и редко, ААВ возникает у детей и сопровождается тяжелыми проявлениями и смертностью, особенно на поздней стадии выявления заболевания. ААВ в большинстве случаев связаны с серологически определяемыми АНЦА. Антитела (АНЦА), направленные против протеиназы-3 (PR3) или миелопероксидазы (МПО) нейтрофильных лейкоцитов, в более чем 80% случаев связаны с ГП или МП. И наоборот, только 50% пациентов с ЭГП являются АНЦА-положительными; и если АНЦА присутствует, то в 75% случаев они направлены против МПО. Применение режимов индукции ремиссии циклофосфамидом (Цф) и высокими дозами кортикостероидов (КС) в течение последних 25 лет перевели ААВ из разряда смертельных в разряд хронически рецидивирующих заболеваний. Исследования по снижению токсичности Цф привели к использованию в поддерживающей терапии более безопасных иммуносупрессантов, таких как метотрексат (МТ) или азатиоприн (АЗА). В дальнейшем стратификация ААВ по степени тяжести выделила пациентов с легким и среднетяжелым течением заболевания, которых лечат с помощью протоколов, не включающих ЦФ. С 2011 г. в первой линии терапии ААВ был введен ритуксимаб, препарат могоклонального анти-СД-20 антитела. В статье обсуждается современная тактика лечения АНЦА-васкулитов у взрослых и детей.

Об авторе

Александр Григорьевич Румянцев
Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России
Россия


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Для цитирования:


Румянцев А.Г. Научно-практический журнал Международной организации Consensus in Pediatrics Лечение васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами. Вопросы гематологии/онкологии и иммунопатологии в педиатрии. 2016;15(2):5-13. https://doi.org/10.24287/1726-1708-2016-15-2-5-13

For citation:


Rumyanstev A.G. Scientific and Practical Journal Global Initiative for Consensus in Pediatrics Management of vasculites associated with anti-neutrophil cytoplasmic antibodies. Pediatric Hematology/Oncology and Immunopathology. 2016;15(2):5-13. (In Russ.) https://doi.org/10.24287/1726-1708-2016-15-2-5-13

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