VEXAS syndrome with severe multisystem involvement: Rapid recovery after splenectomy

Yildirim F., Erdogan M., Yalcin Mutlu M., Akkuzu G., Ozgur D. S., Karaalioglu B., ...Daha Fazla

International Journal of Rheumatic Diseases, cilt.26, sa.3, ss.559-562, 2023 (SCI-Expanded, Scopus) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26 Sayı: 3
  • Basım Tarihi: 2023
  • Doi Numarası: 10.1111/1756-185x.14540
  • Dergi Adı: International Journal of Rheumatic Diseases
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.559-562
  • Anahtar Kelimeler: proteinuria, splenectomy, vasculitis, VEXAS syndrome
  • Marmara Üniversitesi Adresli: Hayır

Özet

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.