rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2026-202-207rsp-3917Research ArticleОПИСАНИЕ СЛУЧАЯCASE DESCRIPTIONПоражение аорты у пациентки с рецидивирующим полихондритомAortic involvement in a patient with relapsing polychondritis. Clinical observationhttps://orcid.org/0000-0003-3183-0464ДибровД. А.DibrovD. A.

Дибров Данил Алексеевич

115522, Москва, Каширское шоссе, 34а

Danil A. Dibrov

115522, Moscow, Kashirskoye Highway, 34A

dibrovd995@gmail.comhttps://orcid.org/0000-0003-3552-2522РешетнякТ. М.ReshetnyakT. M.

115522, Москва, Каширское шоссе, 34а; 125993, Москва, ул. Баррикадная, 2/1, стр. 1

Tatiana M. Reshetnyak

115522, Moscow, Kashirskoye Highway, 34A; 125993, Moscow, Barrikadnaya str., 2/1, building 1

https://orcid.org/0000-0003-3065-4235ДемидоваН. В.DemidovaN. V.

115522, Москва, Каширское шоссе, 34а

Natalia V. Demidova

115522, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0002-9292-6649АлексееваА. В.AlekseevaA. V.

115522, Москва, Каширское шоссе, 34а

Anna V. Alekseeva

115522, Moscow, Kashirskoye Highway, 34A

https://orcid.org/0000-0002-4503-7750БланкЛ. М.BlankL. M.

115522, Москва, Каширское шоссе, 34а

Leonid M. Blank

115522, Moscow, Kashirskoye Highway, 34A

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian FederationФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава РоссииРоссияV.A. Nasonova Research Institute of Rheumatology; Russian Medical Academy of Continuous Professional Education of the Ministry of Healthcare of the Russian FederationRussian Federation202602052026642202207Copyright © Дибров Д.А., Решетняк Т.М., Демидова Н.В., Алексеева А.В., Бланк Л.М., 20262026Дибров Д.А., Решетняк Т.М., Демидова Н.В., Алексеева А.В., Бланк Л.М.Dibrov D.A., Reshetnyak T.M., Demidova N.V., Alekseeva A.V., Blank L.M.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/3917

Рецидивирующий полихондрит (РПХ) – это системное аутоиммунное заболевание, которое сопровождается воспалением и разрушением хрящей разных органов. Поражение аорты при РПХ встречается относительно редко (6,7–9,7%), сопряжено с высоким риском летальных исходов (27,3–41,3%) и оперативных вмешательств (65%). Обычно вовлечение сердца и аорты у пациентов с РПХ выявляется через 5 лет после первых симптомов заболевания, а у 19% протекает бессимптомно. Нет общепринятых стандартов по скринингу, диагностике и терапии поражения аорты при РПХ.

Мы представляем клиническое наблюдение пациентки с РПХ, поражением аорты и развитием аневризмы, которой последовательно проведена терапия циклофосфамидом и ритуксимабом, что позволило достичь контроля над активностью заболевания и снизить дозу глюкокортикоидов.

Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by inflammation and destruction of cartilage in various organs. Aortic involvement in RP is relatively rare, occurring in 6.7–9.7% of cases, and is associated with a high risk of mortality (27.3–41.3%) and surgical interventions (65%). Typically, cardiac and aortic involvement in patients with RP is identified approximately five years after the onset of the first symptoms, with 19% of cases being asymptomatic. There are currently no universally accepted standards for screening, diagnosis, and treatment of aortic involvement in RP.

We present a clinical case of a patient with RP, aortic involvement, and the development of an aneurysm, who underwent sequential therapy with cyclophosphamide and rituximab. This treatment approach allowed for the control of disease activity and a reduction in glucocorticoid dosage.

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