Giant cell arteritis is a primary necrotizing vasculitis characterized by granulomatous involvement of large and medium-sized blood vessels of the aorta with predilection for the extracranial branches of the external carotid artery. Disease severity is in the risk of retinal arteries occlusion that leads a rapid irreversible visual loss.
This condition is frequently accompanied by polymyalgia rheumatica. For diagnosis several modern imaging modalities are of choice - MRI and CT angiography.
Biopsy of temporal artery is recommended in unclear cases. It is essential when the disease is suspected immediate treatment with high doses of corticosteroids must be started.
When corticosteroids are contraindicated or ineffective and for their dose reduction conventional synthetic disease modifying drugs are recommended - usually methotrexate, less then azathioprine, cyclosporine A and cyclophosphamide with inconsistent results. In refractory or relapsing cases administration of biologicals is to be considered.
Monoclonal antibodies to interleukin-6 receptor and interleukin-12/23 seem to be promising.