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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.

ORPHA:397

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
  • OMIM: 187360
  • UMLS: C0039483  C1956391
  • MeSH: D013700
  • GARD: 9615
  • MedDRA: 10018250  10043207

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