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Clinical Case of VEXAS-Negative Recurrent Polychondritis Associated with Lymphoid Tumor

https://doi.org/10.20514/2226-6704-2025-15-5-383-390

EDN: SAPRFQ

Abstract

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.

About the Authors

A. V. Yagoda
Stavropol State Medical University, Department of Hospital Therapy
Russian Federation

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

Stavropol



P. V. Koroy
Stavropol State Medical University, Department of Hospital Therapy
Russian Federation

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

Stavropol



D. P. Kharchenko
Stavropol Regional Clinical Hospital
Russian Federation

Dina P. Kharchenko — Нead of the Rheumatology Department

Stavropol



N. N. Gladkikh
Stavropol State Medical University, Department of Hospital Therapy
Russian Federation

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

Stavropol



I. P. Popova
Stavropol Regional Clinical Hospital
Russian Federation

Inna P. Popova — Doctor of the Therapeutic Department

Stavropol



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Review

For citations:


Yagoda A.V., Koroy P.V., Kharchenko D.P., Gladkikh N.N., Popova I.P. Clinical Case of VEXAS-Negative Recurrent Polychondritis Associated with Lymphoid Tumor. The Russian Archives of Internal Medicine. 2025;15(5):383-390. (In Russ.) https://doi.org/10.20514/2226-6704-2025-15-5-383-390. EDN: SAPRFQ

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ISSN 2226-6704 (Print)
ISSN 2411-6564 (Online)