VEXAS syndrome
MONDO:0026777Mondo
Findings
No curated finding names VEXAS syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
- Inheritance
- Typified by somatic mosaicism
- Onset and course
- Middle age onset
HPO, annotations 2026-09-02
Features
23 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Macrocytic anemiaHPOHP:0001972
- 24 of 25 reported patients
- Recurrent feverHPOHP:0001954
- 23 of 25 reported patients
- Pulmonary infiltratesHPOHP:0002113
- 18 of 25 reported patients
- Chondritis of pinnaHPOHP:0200047
- 16 of 25 reported patients
- Nasal chondritisHPOHP:0033380
- 16 of 25 reported patients
- ThromboembolismHPOHP:0001907
- 11 of 25 reported patients
- ArteritisHPOHP:0012089
- ArthralgiaHPOHP:0002829
- ArthritisHPOHP:0001369
- Autoimmune antibody positivityHPOHP:0030057
- Deep venous thrombosisHPOHP:0002625
- Elevated circulating C-reactive protein concentrationHPOHP:0011227
Show the remaining 11
- Elevated erythrocyte sedimentation rateHPOHP:0003565
- Erythroid dysplasiaHPOHP:0031688
- FatigueHPOHP:0012378
- Inflammatory abnormality of the skinHPOHP:0011123
- Megakaryocyte dysplasiaHPOHP:0031689
- MyelodysplasiaHPOHP:0002863
- Neutrophilic infiltration of the skinHPOHP:0031234
- Night sweatsHPOHP:0030166
Where it sits
Other names
3 names
Resolves to: VEXAS syndrome
- Also called
- vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndromeVEXASVEXAS syndrome, somatic