VEXAS syndrome causing fever of unknown origin

Anna Grosse, Tania Salehi, Mandy Callary, Jane R Hecker and Pravin Hissaria
Med J Aust 2022; 217 (3): . || doi: 10.5694/mja2.51646
Published online: 1 August 2022

A 69‐year‐old man had three hospital admissions from April to July 2021 with persistent fever of unknown origin. The onset was within one week of robotic radical prostatectomy for localised prostate adenocarcinoma. Associated features included diaphoresis, pruritis, anorexia with 15 kg of weight loss, sinus tachycardia, an urticarial rash over the torso and limbs, and chondritis of the left ear. The past medical history included hypertension, dyslipidaemia, gastro‐oesophageal reflux disease, asthma and multiple superficial venous thromboses.

  • 1 Royal Adelaide Hospital, Adelaide, SA
  • 2 SA Pathology, Adelaide, SA


Competing interests:

No relevant disclosures.

  • 1. Beck DB, Ferrada MA, Sikora KA, et al. Somatic mutations in UBA1 and severe adult‐onset autoinflammatory disease. N Engl J Med 2020; 383: 2628‐2638.
  • 2. Grayson PC, Patel BA, Young NS. VEXAS syndrome. Blood 2021; 137: 3591‐3594.
  • 3. Barba T, Jamilloux Y, Durel CA, et al. VEXAS syndrome in a woman. Rheumatology (Oxford) 2021; 60: e402‐e403.
  • 4. Bourbon E, Heiblig M, Gerfaud Valentin M, et al. Therapeutic options in VEXAS syndrome: insights from a retrospective series. Blood 2021; 137: 3682‐3684.
  • 5. Diarra A, Duployez E, Fournier E, et al. Successful allogeneic hematopoietic stem cell transplantation in patients with VEXAS syndrome: a 2‐center experience. Blood Adv 2021; 6: 998‐1003.


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