nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2019-23-5-107-115nefr-1750Research ArticleПРОГРАММА НЕПРЕРЫВНОГО ПОСЛЕДИПЛОМНОГО ОБРАЗОВАНИЯ ПО НЕФРОЛОГИИPROGRAM ON CONTINUOUS POSTGRADUATE EDUCATION ON NEPHROLOGYСтратегия терапии ANCA-ассоциированных ренальных васкулитов у детей и подростковTreatment strategy ANCA-associated renal vasculitides in children and adolescentshttps://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Савенкова Надежда Дмитриевна - профессор, доктор медицинских наук, кафедра факультетской педиатрии, заведующая кафедрой.

194100, Санкт-Петербург, ул. Литовская, д. 2.

Тел.: (812) 416-52-66.

Nadezhda D. Savenkova - MD, PhD, Dr Med Sci, Department of faculty pediatrics, Head.

194100, St-Petersburg, Litovskaya str., 2.

Phone: (812) 416-52-86.

savenkova.n.spb@mail.ruСанкт-Петербургский государственный педиатрический медицинский университетРоссияSaint-Petersburg State Pediatric Medical UniversityRussian Federation201908082019235107115Copyright © Савенкова Н.Д., 20192019Савенкова Н.Д.Savenkova N.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/1750

В статье представлены терминология и классификация в соответствии с International Сhapel Hill Сonsensus Сonference nomenclature of vasculitides (2012), клинико-иммунологические и морфологические проявления, cтратегия терапии и исход ренальных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами (АНЦА) (микроскопического полиангиита, гранулематоза с полиангиитом Вегенера, эозинофильного гранулематоза с полиангиитом Чарга–Стросса) у детей и подростков. Антитела класса IgG к МРО и PR3, гистопатологические изменения в почечных биоптатах считают золотым стандартом в диагностике АНЦА-гломерулонефрита. В соответствии с рекомендациями The European Vasculitis Study Group (EUVAS) у взрослых пациентов АНЦА-ассоциированным васкулитом приведено описание категорий тяжести заболевания: локализованная, ранняя системная, тяжелая, генерализованная, рефрактерная. Представлен алгоритм терапии АНЦА-ассоциированного васкулита, рекомендованный EULAR (2009) для взрослых пациентов и адаптированный для детей L.A. Plumb et al. (2018), который предусматривает дифференцированный подход к индукции ремиссии при локализованной, ранней системной, тяжелой, генерализованной, рефрактерной категориях тяжести и поддерживающей терапии при локализованной, ранней системной, генерализованной категориях, второй линии терапии. В большинстве случаев АНЦА-ассоциированных ренальных васкулитов у детей и подростков именно поражение почек, манифестирующее быстро прогрессирующим гломерулонефритом с острым повреждением почек, определяет тяжесть и прогноз исхода в терминальную уремию. Представляется важным и необходимым в протоколах терапии АНЦА-ассоциированного васкулита включать стратегию ведения до диализа и на диализе быстро прогрессирующего гломерулонефрита с острым повреждением почек у детей и подростков.

The article presents the terminology and classification in accordance with the International Сhapel Hill Сonsensus Сonference nomenclature of vasculitides (2012), clinical, immunological and morphological manifestations, therapy strategy and outcome of Antineutrophil cytoplasmic antibody vasculitides (ANCA) renal associated vasculitis (microscopic polyanghiitis, granulomatosis with Wegener's polyangiitis, eosinophilic granulomatosis with polyangiitis Churg-Strauss) in children and adolescents. IgG class antibodies to MPO and PR3, histopathological changes in renal biopsy specimens are considered the gold standard in the diagnosis of ANCA-glomerulonephritis. Following the recommendations of The European Vasculitis Study Group (EUVAS) in adult patients, ANCA-associated vasculitis describes the categories of disease severity: localized, early systemic, severe, generalized, refractory. An algorithm for the treatment of ANCA-associated vasculitis, recommended by EULAR (2009) for adult patients and adapted for children of L.A. Plumb et al (2018), which provides for a differentiated approach to the induction of remission in localized, early systemic, severe, generalized, refractory categories of severity and supportive therapy in localized, early systemic, generalized categories, second-line therapy. In most cases of ANCA-associated renal vasculitis in children and adolescents, it is kidney damage that manifests rapidly progressive glomerulonephritis with acute kidney damage, determines the severity and prognosis of outcome in terminal uremia. It seems important and necessary in the treatment protocols of ANCA-associated vasculitis to include a strategy for pre-dialysis and dialysis of rapidly progressive glomerulonephritis with acute kidney damage in children and adolescents.

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