VEXAS Syndrome with an Improvement of Chest Shadows without Immunosuppressive Therapy

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an acquired autoinflammatory disease caused by UBA1 variants. We report an 86-year-old man with high C-reactive protein levels, bicytopenia, and bilateral chest shadows. Bronchoalveolar lavage revealed an elevated lymphocyte count. Bone marrow vacuoles and a UBA1 (c.122T>C p.Met41Thr) variant with a high variant allele frequency (VAF) confirmed VEXAS. Although he refused treatment, chest shadows improved without immunosuppressive therapy and remained stable for two months, despite the persistence of systemic inflammatory symptoms and elevated C-reactive protein levels. This case highlights the clinical heterogeneity of VEXAS syndrome, demonstrating that pulmonary involvement can be self-limiting despite high VAF and atypical lavage findings, suggesting careful consideration of the treatment timing.