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

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.