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ORPHA:397Disease
Also called Horton disease · Temporal arteritis
What it is
A rare large vessel vasculitis (LVV) characterized by vasculitis predominantly involving the arteries originating from the aortic arch and the extracranial branches of the carotid arteries. Clinical manifestations are variable, the predominant cranial phenotype is characterized by headache, jaw claudication, scalp tenderness and visual symptoms and the predominant LVV type by constitutional symptoms, polymyalgia rheumatica and occasionally limb ischemia. Overlaps between these two phenotypes are common.
Key facts
- Prevalence
- 1-5 / 10 000 (Europe)
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial
- 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%
10Common30–79%
18- Abnormal systemic blood pressure
- Alopecia
- Anemia
- Arthritis
- Blurred vision
- Carotidynia
- Depression
- Elevated circulating calprotectin concentration
- Elevated circulating osteopontin level
- Elevated erythrocyte sedimentation rate
- Hyperfibrinogenemia
- Increased alpha-globulin
- Increased circulating interleukin 6 concentration
- Jaw claudication
- Malaise
- Ophthalmoparesis
- Polyarticular arthritis
- Visual impairment
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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