mrjСовременная ревматологияModern Rheumatology Journal1996-70122310-158XIMA-PRESS, LLC10.14412/1996-7012-2023-2-7-15mrj-1404Research ArticleЛЕКЦИЯLECTUREДифференциальная диагностика локальной формы гранулематоза с полиангиитом: поражение органа зрения и органа слуха (часть 2)Differential diagnosis of a local form of granulomatosis with polyangiitis: ocular and ear involvement (part 2)https://orcid.org/0000-0002-4846-5531ЕгороваО. Н.EgorovaO. N.

Ольга Николаевна Егорова

115522, Москва, Каширское шоссе, 34А

Olga Nikolaevna Egorova

34A, Kashirskoe Shosse, Moscow 115522

onegorova@yandex.ruhttps://orcid.org/0000-0001-8864-7623ХарламоваЕ. Н.KharlamovaE. N.

115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522

https://orcid.org/0000-0001-9933-5350ТарасоваГ. М.TarasovaG. M.

115522, Москва, Каширское шоссе, 34А

34A, Kashirskoe Shosse, Moscow 115522

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation202322042023172715Copyright © Егорова О.Н., Харламова Е.Н., Тарасова Г.М., 20232023Егорова О.Н., Харламова Е.Н., Тарасова Г.М.Egorova O.N., Kharlamova E.N., Tarasova G.M.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/1404

Гранулематоз с полиангиитом (ГПА) – первичный васкулит, ассоциированный с антинейтрофильными цитоплазматическими антителами, характеризующийся некротизирующим васкулитом  с преимущественным вовлечением мелких  сосудов различной локализации  и некротизирующим гранулематозным воспалением с многогранными клиническими  проявлениями. ГПА остается одним из самых тяжелых и прогностически неблагоприятных системных васкулитов.  Во второй части статьи представлены данные о дифференциальной диагностике дебюта заболевания с поражения органа зрения и органа слуха, которая требует междисциплинарного подхода и взаимодействия между врачами разных специальностей. При рефрактерном течении локальной формы ГПА целесообразна иммуносупрессивная терапия, включающая глюкокортикоиды и ритуксимаб или циклофосфамид.

Granulomatosis with polyangiitis (GPA) is a primary vasculitis associated with antineutrophil cytoplasmic antibodies, characterized by necrotizing vasculitis with predominant involvement of small vessels of various localization and necrotizing granulomatous inflammation with multiple clinical manifestations. GPA remains one of the most severe and prognostically unfavorable systemic vasculitis. The second part of the article presents data on the differential diagnosis of the onset of the disease with the ocular and ear involvement, which requires an interdisciplinary approach and interaction between doctors of different specialties. In the refractory course of the local form of GPA, immunosuppressive therapy, including glucocorticoids and rituximab or cyclophosphamide, is advisable.

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