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Broadening the clinical spectrum of giant cell arteritis: from the classic cranial to the predominantly extracranial pattern of the disease.

INTRODUCTION: Giant cell arteritis (GCA) is a large vessel (LV) vasculitis thataffects people aged 50 years and older. Classically, GCA was considered adisease that involved branches of the carotid artery. However, the advent ofnew imaging techniques has allowed us to reconsider the clinical spectrum ofthis vasculitis.

AREASCOVERED: The review describesclinical differences between patients with the cranial GCA and those with apredominantly extracranial LV-GCA disease pattern. It highlights differences inthe frequency of positive temporal artery biopsy depending on the predominantdisease pattern and emphasizes the relevance of imaging techniques to identifypatients with LV-GCA without cranial ischemic manifestations. The review showsthat so far there are no well-established differences in genetic predispositionto GCA regardless of the predominant phenotype.

EXPERTCOMMENTARY: The large branches ofthe extracranial arteries are frequently affected in GCA. Imaging techniquesare useful to identify the presence of 'silent' GCA in people presentingwith polymyalgia rheumatica or with nonspecific manifestations. Whether thesetwo different clinical presentations of GCA constitute a continuum in theclinical spectrum of the disease or whether they may be related but aredefinitely different conditions needs to be further investigated.

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