pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2022-4101pulmo-4101Research ArticleОБЗОРЫREVIEWЭозинофильный гранулематоз с полиангиитом: этиопатогенез, классификация и клинические фенотипыEosinophilic granulomatosis with polyangiitis: etiopathogenesis, classification and clinical phenotypeshttps://orcid.org/0000-0003-3672-9242АнаевЭ. Х.AnaevE. Kh.

Анаев Эльдар Хусеевич – доктор медицинских наук, профессор кафедры пульмонологии.

117997, Москва, ул. Островитянова, 1

Eldar Kh. Anaev - Doctor of Medicine, Professor, Department of Pulmonology.

Ul. Ostrovityanova 1, Moscow, 117997; tel.: (499) 780-08-43

el_anaev@hotmail.comhttps://orcid.org/0000-0001-6050-724XБелевскийА. С.BelevskiyA. S.

Белевский Андрей Станиславович – доктор медицинских наук, профессор, заведующий кафедрой пульмонологии.

117997, Москва, ул. Островитянова, 1; тел.: (495) 965-09-27

Andrey S. Belevskiy - Doctor of Medicine, Professor, Head of the Department of Pulmonology.

Ul. Ostrovityanova 1, Moscow, 117997; tel.: (495) 965-09-27

pulmobas@yandex.ruhttps://orcid.org/0000-0002-1562-6386КняжескаяН. П.KniajeskaiaN. P.

Княжеская Надежда Павловна – кандидат медицинских наук, доцент кафедры пульмонологии факультета дополнительного профессионального образования.

117997, Москва, ул. Островитянова, 1; тел.: (499) 780-08-43

Nadezhda P. Knyazheskaya, Candidate of Medicine, Associate Professor, Head of the Department of Pulmonology, Faculty of Additional Professional Education.

Ul. Ostrovityanova 1, Moscow, 117997; tel.: (499) 780-08-43

kniajeskaia@mail.ruФедеральное государственное автономное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияPirogov Russian National Research Medical University (Pirogov Medical University), Ministry of Health of the Russian FederationRussian Federation202309062023333393400Copyright © Анаев Э.Х., Белевский А.С., Княжеская Н.П., 20232023Анаев Э.Х., Белевский А.С., Княжеская Н.П.Anaev E.K., Belevskiy A.S., Kniajeskaia N.P.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/4101

Эозинофильный гранулематоз с полиангиитом (ЭГПА) – редкое системное заболевание, которое относится как к гиперэозинофильным состояниям, так и к васкулитам, ассоциированным с антинейтрофильными цитоплазматическими антителами (АНЦА), и характеризуемое гранулематозным воспалением. Патогенез ЭГПА остается неясным. Вероятно, это заболевание является Th2-опосредованным, а эозинофилия крови и тканей – его главный диагностический критерий. Отличительные признаки и основные эффекторы повреждения органов в случае ЭГПА – это некротизирующий васкулит мелких и средних сосудов, ассоциированный с бронхиальной астмой, и эозинофильная пролиферация. Повреждение эндотелия и воспаление сосудов при ЭГПА вызвано АНЦА путем активации циркулирующих нейтрофилов. В соответствии с обнаружением АНЦА описаны 2 клинических фенотипа заболевания: АНЦАнегативный с проявлениями гиперэозинофилии (например, легочные инфильтраты и кардиомиопатия) и АНЦА-позитивный с клинической картиной васкулита (например, гломерулонефрит, пурпура и множественный мононеврит), подтвержденные по результатам гистологических и геномных исследований. Однако в клинической практике эти два сосуществующих механизма разделить невозможно.

Целью работы явилось информирование специалистов о современных представлениях об эозинофильных и АНЦА-опосредованных аспектах патогенеза, классификации и клинических фенотипов ЭГПА и перспективах будущих исследований.

Заключение. Основу развития ЭГПА составляет эозинофильная дисфункция, при которой у пациентов с генетически детерминированной предрасположенностью к распознаванию антигена АНЦА и выявленных аллелях HLA-DQ (Human Leukocyte Antigen DQ) вырабатываются аутоантитела к миелопероксидазе, а позднее развивается аберрантный аутоиммунный процесс. Таким образом, для выявления патогенетических механизмов и молекулярной характеристики клинических фенотипов ЭГПА необходимы дальнейшие комплексные постгеномные исследования. При выявлении молекулярных эндотипов и идентификации новых биомаркеров активности и терапевтических мишеней могут улучшиться диагностика ЭГПА и результаты лечения.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease that can be classified as both a hypereosinophilic condition and an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and is characterized by granulomatous inflammation. The pathogenesis of EGPA is not completely understood. It is likely that this disease is Th2-mediated, and blood and tissue eosinophilia serves as the main diagnostic criterion. The hallmarks and main effectors of organ damage in EGPA include asthma-associated necrotizing vasculitis of small-to-medium vessels and eosinophilic proliferation. Endothelial injury and vascular inflammation in EGPA is caused by ANCA via activation of circulating neutrophils. Two clinical phenotypes of the disease have been described based on the detection of ANCA: ANCA-negative with manifestations of hypereosinophilia (for example, pulmonary infiltrates and cardiomyopathy) and ANCA-positive with clinical signs of vasculitis (for example, glomerulonephritis, purpura, and mononeuritis multiplex). Both phenotypes were confirmed by histological and genomic research. However, these two coexisting mechanisms cannot be separated in clinical practice.

The aim of the article is to present current knowledge of eosinophilic and ANCA-mediated aspects of the pathogenesis, classification and clinical phenotypes of EGPA, and consider prospects for future research.

Conclusion. The development of EGPA is based on eosinophilic dysfunction. This dysfunction means that patients with a genetically determined predisposition to recognize the ANCA antigen and with HLA-DQ (human leukocyte antigen DQ) alleles produce anti-myeloperoxidase autoantibodies and later develop an aberrant autoimmune process. Further comprehensive post-genomic studies are needed to identify the pathogenetic mechanisms and characterize molecular features of EGPA clinical phenotypes. The elaboration of molecular endotypes will lead to the identification of new activity biomarkers and therapeutic targets that can improve the diagnosis of EGPA and the treatment outcomes.

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