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Giant cell arteritis

ORPHA:397· ICD-10 M31.6

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.

Prevalence
1-9 / 100 000
Inheritance
Multigenic/multifactorial
Age of onset
Adult