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VEXAS syndrome with severe multisystem involvement: Rapid recovery after splenectomy.

Fatih YildirimMustafa ErdoganMelek Yalcin MutluGamze AkkuzuDuygu Sevinç OzgurBilgin KaraaliogluMesut AyerCemal Beş
Published in: International journal of rheumatic diseases (2022)
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined disorder in which treatment is still unclear. Herein, a patient with VEXAS syndrome who had atypical findings and an interesting treatment course is presented as a case report. He had fatigue, recurrent fever, pulmonary infiltrates, proteinuria, anemia, leucopenia, transient skin rush and increased acute phase reactants. The patient, who could not tolerate corticosteroid tapering, recovered rapidly after diagnostic splenectomy and the pathological examination of the spleen revealed significant findings.
Keyphrases
  • case report
  • chronic kidney disease
  • pulmonary hypertension
  • physical activity
  • early onset
  • brain injury
  • subarachnoid hemorrhage