VEXAS syndrome with severe multisystem involvement: Rapid recovery after splenectomy.
VEXAS syndrome
proteinuria
splenectomy
vasculitis
Journal
International journal of rheumatic diseases
ISSN: 1756-185X
Titre abrégé: Int J Rheum Dis
Pays: England
ID NLM: 101474930
Informations de publication
Date de publication:
Mar 2023
Mar 2023
Historique:
revised:
11
10
2022
received:
16
07
2022
accepted:
11
12
2022
pubmed:
23
12
2022
medline:
4
3
2023
entrez:
22
12
2022
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 36544349
doi: 10.1111/1756-185X.14540
doi:
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
559-562Informations de copyright
© 2022 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.
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