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Extensive Multiple Organ Involvement in VEXAS Syndrome
- Source :
- Arthritis & Rheumatology. 73:1896-1897
- Publication Year :
- 2021
- Publisher :
- Wiley, 2021.
-
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.
- Subjects :
- Optic perineuritis
Pathology
medicine.medical_specialty
medicine.diagnostic_test
business.industry
Somatic cell
Immunology
Magnetic resonance imaging
Inflammation
medicine.disease
Rheumatology
Recurrent fever
medicine
Immunology and Allergy
Organ involvement
Autoinflammatory disease
medicine.symptom
business
Scleritis
Subjects
Details
- ISSN :
- 23265205 and 23265191
- Volume :
- 73
- Database :
- OpenAIRE
- Journal :
- Arthritis & Rheumatology
- Accession number :
- edsair.doi...........0ec514e5a8266ea138134ad4de4aa329