rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2020-550-559rsp-2956Research ArticleОБЗОРЫ И ЛЕКЦИИREVIEWS AND LECTURESКлинико-иммунологические варианты АНЦА-ассоциированного системного васкулита: взгляд на феномен гиперпродукции IgG4. Обзор литературы и собственное наблюдениеClinical and immunological variants of ANCA-associated systemic vasculitis: a look at the phenomenon of IgG4 overproduction. Literature review and own observationshttps://orcid.org/0000-0003-2641-9785БекетоваТ. В.BeketovaT. V.

Бекетова Татьяна Валентиновна

115522, Москва, Каширское шоссе, 34а

Tatiana V. Beketova

115522, Moscow, Kashirskoe highway, 34a

 

tvbek22@rambler.ruКокосадзеН. В.KokosadzeN. V.

115478, Москва, Каширское шоссе, 23

N.V. Kokosadze

115478, Moscow, Kashirskoe highway, 23

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian FederationНИИ клинической онкологии ФГБУ «НМИЦ онкологии им. Н. Н. Блохина» Минздрава РоссииРоссияResearch Institute of Clinical Oncology of the N.N. Blokhin Oncology CenterRussian Federation202009112020585550559Copyright © Бекетова Т.В., Кокосадзе Н.В., 20202020Бекетова Т.В., Кокосадзе Н.В.Beketova T.V., Kokosadze N.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2956

Системные васкулиты, ассоциированные с антинейтрофильными цитоплазматическими антителами, представляют собой многофакторный процесс, характеризующийся вариабельностью эпитопной специфичности данных антител и разнообразием клинических фенотипов заболевания. В последние годы наблюдается рост интереса к сочетанию системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами, и феномена гиперпродукции IgG4, варьирующей при них в широких пределах, от продукции антинейтрофильных цитоплазматических антител подкласса IgG4 неопределенного значения, присутствия IgG4 позитивных плазматических клеток в очагах иммуновоспалительного поражения у пациентов с несомненным диагнозом системного васкулита, ассоциированного с антинейтрофильными цитоплазматическими антителами, до типичных клинических проявлений IgG4-связанного заболевания. Представлено собственное клиническое наблюдение и проанализированы объединенные данные литературы, включившие 35 случаев сочетания системного васкулита, ассоциированного с антинейтрофильными цитоплазматическими антителами, и IgG4-связанной патологии, свидетельствующие о существующих различиях в клинических проявлениях классического IgG4-связанного заболевания и при его сочетании с системным васкулитом, ассоциированным с антинейтрофильными цитоплазматическими антителами.

Накопленные в настоящее время данные позволяют обсуждать выделение особого клинико-иммунологического варианта системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами, с гиперпродукцией IgG4, характеризующегося сочетанием клинических проявлений системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами, и клинических и/или гистологических признаков IgG4 связанной патологии. Важно подчеркнуть, что выделение фенотипов системных васкулитов, ассоциированных с антинейтрофильными цитоплазматическими антителами, в перспективе может иметь значение для персонифицированного выбора тактики лечения пациентов.

Systemic vasculitis (SV) associated with anti-neutrophilic cytoplasmic antibodies is a multifactorial process characterized by the variability of the epitope specificity of anti-neutrophilic cytoplasmic antibodies and the diversity of clinical phenotypes of the disease. In recent years, there has been an increasing interest in the combination of AAV and the phenomenon of IgG4 hyperproduction, which varies widely within AAV, from anti-neutrophilic cytoplasmic antibodies products of IgG4 subclass of undetermined significance, the presence of IgG4 positive plasma cells in the foci of immunoinflammatory lesions in patients with a definite diagnosis of AAV, to the typical clinical manifestations of an IgG4-related disease (IgG4-RD). We introduce own clinical case and analyze the combined data of the literature, which included 35 cases of a combination of AAV and IgG4-related pathology, indicating existing differences in the clinical manifestations of classical IgG4-RD and when combined with AAV. The currently accumulated data allows us to discuss the allocation of a specific clinical and immunological variant of AAV with IgG4 hyperproduction, characterized by a combination of clinical manifestations of AAV and clinical and / or histological signs of IgG4-related pathology. It is important to emphasize that the allocation of phenotypes of AAV in the future may be important for the personalized choice of treatment tactics for patients.

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