avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2025-15-5-383-390SAPRFQavk-2083Research ArticleРАЗБОР КЛИНИЧЕСКИХ СЛУЧАЕВANALYSIS OF CLINICAL CASESКлинический случай VEXAS-негативного рецидивирующего полихондрита, ассоциированного с лимфоидной опухольюClinical Case of VEXAS-Negative Recurrent Polychondritis Associated with Lymphoid Tumorhttps://orcid.org/0000-0002-5727-1640ЯгодаА. В.YagodaA. V.

Ягода Александр Валентинович — д.м.н., профессор, заслуженный деятель науки РФ, заведующий кафедрой госпитальной терапии 

Ставрополь

Alexander V. Yagoda — Doctor of Medical Sciences, Professor, Head of the Department of Hospital Therapy

Stavropol

alexander.yagoda@gmail.comhttps://orcid.org/0000-0001-6392-8461КоройП. В.KoroyP. V.

Корой Павел Владимирович — д.м.н., профессор, профессор кафедры госпитальной терапии

Ставрополь

Pavel V. Koroy — MD, PhD, Professor, Professor of Department of Hospital Therapy

Stavropol

ХарченкоД. П.KharchenkoD. P.

Харченко Дина Петровна — заведующая ревматологическим отделением

Ставрополь

Dina P. Kharchenko — Нead of the Rheumatology Department

Stavropol

https://orcid.org/0000-0003-4510-3604ГладкихН. Н.GladkikhN. N.

Гладких Наталья Николаевна — д.м.н., профессор, профессор кафедры госпитальной терапии 

Ставрополь

Natalia N. Gladkikh — MD, PhD, Professor, Professor of Department of Hospital Therapy

Stavropol

ПоповаИ. П.PopovaI. P.

Попова Инна Петровна — врач терапевтического отделения

Ставрополь

Inna P. Popova — Doctor of the Therapeutic Department

Stavropol

ФГБОУ ВО «Ставропольский государственный медицинский университет» Минздрава РоссииРоссияStavropol State Medical University, Department of Hospital TherapyRussian FederationГБУЗ СК «Ставропольская краевая клиническая больница»РоссияStavropol Regional Clinical HospitalRussian Federation202525092025155383390Copyright © Ягода А.В., Корой П.В., Харченко Д.П., Гладких Н.Н., Попова И.П., 20252025Ягода А.В., Корой П.В., Харченко Д.П., Гладких Н.Н., Попова И.П.Yagoda A.V., Koroy P.V., Kharchenko D.P., Gladkikh N.N., Popova I.P.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/2083

Представлено наблюдение лимфоидной опухоли, скрывающейся под «маской» рецидивирующего полихондрита с высокой активностью воспалительного процесса и ограниченным ответом на лечение. Помимо поражения ушной раковины, заболевание сопровождалось неэрозивным недеформирующим артритом, теносиновитом ахиллова сухожилия с его разрывом, эписклеритом, хондритом носа, поражением кожи в виде нейтрофильного дерматоза Свита, дилатацией восходящего отдела аорты, субклиническими признаками поражения лёгких и перикарда, а также развитием макроцитарной анемии при нормальном содержании в крови витамина В12 и фолиевой кислоты, отсутствием лабораторных проявлений аутоиммунизации. Несмотря на то, что рецидивирующий полихондрит с подобными клинико-лабораторными признаками может быть частью фенотипа недавно описанного аутовоспалительного синдрома взрослых (VEXAS), определение его генетического маркера дало отрицательный результат.

A clinical case of lymphoid tumor hiding under the «mask» of recurrent polychondritis with high activity of inflammatory process and limited response to treatment is presented. In addition to auricle damage, the disease was accompanied by non-erosive non-deforming arthritis, tenosynovitis of the Achilles tendon with its rupture, episcleritis, nasal chondritis, skin lesions in the form of Sweet neutrophilic dermatosis, ascending aorta dilation, subclinical signs of lung and pericardial damage, and development of macrocytic anemia with normal blood levels of vitamin B12 and folic acid, absence of laboratory manifestations of autoimmunity. Despite the fact that recurrent polychondritis with similar clinical and laboratory signs may be part of the phenotype of recently described adult autoinflammatory syndrome (VEXAS), the determination of its genetic marker gave a negative result.

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