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ORPHA:3287Disease
What it is
A rare predominantly large-vessel vasculitis that is characterized by affected aorta and its major branches, but also other large vessels, causing stenosis, occlusion, or aneurysm.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adolescent, Adult
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
9Common30–79%
24- Abnormal aortic valve morphology
- Absent pulse
- Anemia
- Anorexia
- Arthritis
- Ascending tubular aorta aneurysm
- Asymmetric blood pressure between arms
- Blood pressure substantially higher in legs than arms
- Carotidynia
- Chest pain
- Elevated erythrocyte sedimentation rate
- Gangrene
- Headache
- Hypertension
- Increased inflammatory response
- Intermittent claudication
- Migraine
- Muscle weakness
- Myalgia
- Myocardial infarction
- Pulmonary arterial hypertension
- Renal artery stenosis
- Seizure
- Skin ulcer
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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