Extensive multiple organ involvement in VEXAS syndrome

• Takahashi Noriyuki

• Takeichi Takuya

• Nishida Tetsuya

• Takahashi Yasuhiro

• Sato Juichi

• Yamamura Masahiro

• Ogi Tomoo

• Akiyama Masashi

Abstract

A 55-year-old Japanese man was diagnosed with VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, which is a newly documented adult-onset autoinflammatory disease caused by somatic UBA1 mutations [1], after four years of symptoms. He had suffered from recurrent fever after the onset of systemic arthralgia, scleritis, periorbital/orbital inflammation, optic perineuritis (A, B, T1-weighted contrast-enhanced magnetic resonance imaging in C and D, arrow in D), and myelodysplastic syndrome.

First published: 21 April 2021

Journal

• Arthritis & Rheumatology

  Arthritis & Rheumatology 73 (10), 1896-1897, 2021-10

  Wiley

Keywords

• VEXAS syndrome
• VEXAS症候群
• 自己炎症性疾患
• Autoinflammatory disease

Details

• CRID: 1050007515086471680
• NII Article ID: 120007126470
• DOI: 10.1002/art.41775
• ISSN: 23265205
• ISSN: 23265191
• HANDLE: 2237/0002000161
• Web Site: https://nagoya.repo.nii.ac.jp/records/2000161
• Web Site: https://onlinelibrary.wiley.com/doi/pdf/10.1002/art.41775
• Web Site: https://onlinelibrary.wiley.com/doi/full-xml/10.1002/art.41775
• Text Lang: en
• Article Type: journal article
• Data Source:
  • IRDB
  • Crossref
  • CiNii Articles
  • KAKEN