rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2023-62-69rsp-3279Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESКлинико-иммунологические варианты АНЦА-ассоциированного системного васкулита: в фокусе интерстициальный фиброз легких при микроскопическом полиангиите с гиперпродукцией антител к миелопероксидазе и ревматоидного фактора. Собственное наблюдение и обзор литературыClinical and immunological variants of ANCA-associated systemic vasculitis: a look at the pulmonary fibrosis in microscopic polyangiitis with anti-myeloperoxidase antibodies and rheumatoid factor. Literature review and own observationhttps://orcid.org/0000-0003-2641-9785БекетоваТ. В.BeketovaT. V.

Бекетова Татьяна Валентиновна

115522, Российская Федерация, Москва, Каширское шоссе, 34а121356, Российская Федерация, Москва, ул. МаршалаТимошенко, 15107023, Российская Федерация, Москва, ул. Большая Семёновская, 38 

115522, Russian Federation, Moscow, Kashirskoye Highway, 34A 

121359, Russian Federation, Moscow, Marshala Timoshenko str., 15 

107023, Russian Federation, Moscow, Bolshaya Semyonovskaya str., 38 

tvbek22@rambler.ruhttps://orcid.org/0000-0001-7247-545XГоловинаО. А.GolovinaO. A.

115522, Российская Федерация, Москва, Каширское шоссе, 34а

115522, Russian Federation, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0003-3057-9175АвдееваА. С.AvdeevaA. S.

115522, Российская Федерация, Москва, Каширское шоссе, 34а

115522, Russian Federation, Moscow, Kashirskoye Highway, 34A

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГБУ «Центральная клиническая больница с поликлиникой» Управления делами Президента Российской Федерации; ФГАОУ ВО «Московский политехнический университет»РоссияV.A. Nasonova Research Institute of Rheumatology; Central Clinical Hospital of the Administrative Directorate of the President of the Russian Federation; Moscow Polytechnic UniversityRussian FederationФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation2023030320236116269Copyright © Бекетова Т.В., Головина О.А., Авдеева А.С., 20232023Бекетова Т.В., Головина О.А., Авдеева А.С.Beketova T.V., Golovina O.A., Avdeeva A.S.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/3279

В зависимости от эпитопной специфичности антинейтрофильных цитоплазматических антител (АНЦА) и наличия или отсутствия гранулематозной воспалительной реакции пациенты с АНЦА-ассоциированными системными васкулитами (СВ) демонстрируют вариабельность клинического течения и прогноза заболевания, что определяет необходимость выделения клинико-иммунологических фенотипов АНЦА-СВ и персонифицированного подхода к лечению. Особенностью микроскопического полиангиита (МПА) с гиперпродукцией антител к миелопероксидазе (МП) является относительно высокая частота поражения легких в манифестную фазу болезни с возможностью развития интерстициального фиброза легких. Представлен случай интерстициального фиброза легких при МПА с гиперпродукцией антител к МП и ревматоидного фактора. Обсуждаются данные литературы и подчеркнута важность включения АНЦА-СВ в круг диагностического поиска у пациентов с дебютом интерстициальной пневмонии и ранним артритом. При этом требуется планомерное обследование пациентов для выявления патогномоничных клинических признаков АНЦАСВ, а также его вариантов, протекающих бессимптомно. Иммунологическое обследование с обязательным определением эпитопной специфичности АНЦА следует выполнять до назначения иммуносупрессантов. Рассматриваются возможности терапии, включая применение ритуксимаба, микофенолата мофетила и нинтеданиба.

Depending on the epitope specificity of antineutrophil cytoplasmic antibodies (ANCA) and the presence or absence of a granulomatous inflammation. Patients with ANCA-associated vasculitis (AAV) show variability in clinical manifestations and prognosis depending on the epitope specificity of ANCA and the presence or absence of granulomatous inflammation. In this regard, it is important to identify the clinical and immunological phenotypes of AAV and a personalized approach to treatment. Microscopic polyangiitis (MPA) with antibodies to myeloperoxidase (aMPO) has a relatively high incidence of lung involvement and pulmonary fibrosis. We present our own clinical case of pulmonary fibrosis in MPA with aMPO and rheumatoid factor. Literature data are discussed. In cases of early arthritis and interstitial pneumonia, the possibility of AAV should be considered, a detailed examination is necessary to identify pathognomonic signs of AAV, including asymptomatic ones. Before prescribing immunosuppressants, the epitope specificity of ANCA should be determined. Treatment with rituximab, mycophenolate mofetil and nintedanib is discussed.

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