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Table 1 Systemic manifestations of VEXAS syndrome in a case of a 76-year-old man

From: Recurrent superior orbital fissure syndrome associated with VEXAS syndrome

Organ system

Symptoms

Respiratory

Recurrent chest infections

Pleural effusions

Pulmonary hypertension

Cardiovascular

Heart failure with preserved ejection fraction

Haematological

Bilateral pulmonary emboli

Saphenous vein occlusion

Iron deficiency anemia

Renal

Hyponatremia

Microalbuminuria

Skin

Widespread erythematous plaques

Leucocytoclastic vasculitis

Musculoskeletal

Muscle wasting

Globally stiff movements around the spine and hips

Other

Fatigue

Weight loss