Table 4. Proposed algorithm to identify patients with VEXAS syndrome.

Demographic	Clinical	Coexistence with	Laboratory Findings	Genetic Findings
Age older age group	Unexplained recurrent fever	Relapsing polychondritis resistant to treatment and/or with a different clinical course	Increase serum cytokines(IL-1, IL-6, IL-17, TNF-alpha)	UBA1 mutation
Gender predominantly male	Recurrent polychondritis (ear-nose)	Treatment-resistant vasculitis, which is beyond the classical knowledge	Unexplained very high CRP/ ESR
Population Caucasian	Inflammatory arthritis usually involving large joints of the lower extremities	Spondylarthritis or connective tissue diseases (ex. SLE)	Macrocytic anemia
	Vasculitis in the presence of simultaneous involvement of vessels of different diameters	Hematological malignancy (MDS) with signs of autoimmune disease	Presence of typical vacuoles in bone marrow biopsy
	Eye involvement treatment-resistant scleritis/ episcleritis/retinal vasculitis
	Severe, treatment-resistant skin lesions(inc. leucocytoclastic vasculitis and Sweet s/m)
	Lung involvement neutrophilic alveolitis
	Kidney involvement interstitial nephritis

CRP:C-reactive protein; ESR:erythrocyte sedimentation rate; IL-1:interleukine-1; IL-6:interleukine-6; IL-17:interleukine-17; MDS: myelodisplastic syndrome; SLE:systemic lupus erythematosus;TNF-alpha:tumor necrosis factor-alpha; UBA1: Ubiquitin-like modifier-activating enzyme 1.