Table 2 Summary of clinical characteristics of VEXAS syndrome
Organ | Inflammatory symptoms |
|---|---|
Constitutional | Fever Fatigue Weight loss Night sweats |
Mucocutaneous | Panniculitis-like nodular lesions Urticarial vasculitis Livedo reticularis Erythema nodosum Neutrophilic dermatosis |
Cartilage | Ear and nose chondritis |
Lung | Cough and shortness of breath Pulmonary infiltrates Ground-glass opacities Organizing pneumonia Pleural effusion |
Musculoskeletal | Arthralgia Arthritis |
Vessels | Vasculitis Thrombotic manifestations (Pulmonary embolism, deep venous thrombosis) |
Liver/spleen | Hepatosplenomegaly |
Heart | Myocarditis |
Ophthalmologic | Episcleritis Periorbital edema |
Lymphoproliferation | Lymphadenopathy |
Other | Macrophage Activation Syndrome [30, 31] Optic neuritis Vestibular dysfunction |