VEXAS Syndrome Presenting as Waxing and Waning Large Vein Phlebitis and Periarteritis With Minimal Vacuolation: Expanding the Clinical Spectrum

Key Points A novel clinical phenotype of VEXAS in the form of periarteritis and large vein phlebitis with a waxing, waning course has been reported. Absence of prominent vacuolations in the myeloid precursors does not rule out a diagnosis of VEXAS. Genetic testing should be performed in suspected cases of VEXAS in an appropriate clinical setting, even in the absence of conspicuous bone marrow findings.