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Giant cell arteritis

Disease definition

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.


Classification level: Disorder
  • Synonym(s):
    • Horton disease
    • Temporal arteritis
  • Prevalence: 1-5 / 10 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: M31.6
  • ICD-11: 4A44.2
  • OMIM: 187360
  • UMLS: C0039483
  • MeSH: D013700
  • GARD: 9615
  • MedDRA: 10018250  10043207

Detailed information

General public


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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