Background/Purpose: Giant cell arteritis (GCA) represents the most common primary vasculitis of the elderly, that affect large and medium-sized arterial vessels. Growing data shown that almost one-third of the patients with GCA develop serious peripheral vascular complications during long-term follow up, and it seems that unrecognized extra-cranial involvement may be even more common. 

The primary aim of this study was to explore if there are different clinical entities in a cohort of patients with GCA (i.e. symptoms compatible with cranial GCA and large-vessel GCA); a secondary aim was to evaluate long-term outcome of GCA patients with at list 5 years of follow-up.

Methods: Two hundred and ten GCA patients (34 males and 176 females, mean ± SD age 77±8 years) were retrospectively studied. Clinical symptoms at disease onset and during the follow-up, time delay until diagnosis, as well as laboratory findings at the time of diagnosis, therapeutic approach were retrospective evaluated. In order to characterized the different disease entities, overall clinical symptoms were evaluated by means of a spectrum analysis. Moreover, we evaluated long-term outcomes in patients with a minimum follow-up of 5 years. We have defined as disease flare any further clinical manifestation compatible with the clinical spectrum of GCA and/or an increase of ESR ≥ 40 mm/hour, not otherwise justifiable, that required higher doses or new introduction of GC therapy.

Results: The most frequent clinical manifestations presented at the onset included: new onset headache and/or scalp pain 77%, constitutional symptoms 46%, jaw claudicatio 36%, vision loss 34%, abnormal temporal artery on examination 32%, dizziness 29%, neuropsychiatric symptoms 29%, cough not otherwise justifiable 10%, cerebrovascular accidents 6% and hearing loss 5%. Irreversible blindness was reported in 7% of patients, mainly due to a latency period between onset and treatment of ≥ 2 months. Temporal artery biopsy was performed in 160/210 of patients, resulting positive in 58%. Globally, about 26% of subjects (55, M/F: 9/46, mean ± SD age 71±7 years) was characterized by a clinical profile compatible with extra-cranial GCA. In all case, extra-cranial involvement was confirmed by (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and/or angiography. Moreover, patients who presented symptoms compatible with large-vessel GCA were characterised by a more relapsing course compared with patients with cranial involvement GCA profile.

Conclusion: According literature data, our results confirm that GCA may be composed by more than one single entity. Extra cranial involvement due to GCA must be must be promptly recognized, because it probably may require a different prognostic and therapeutic approach.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/may-giant-cell-arteritis-be-characterized-by-different-clinical-entity-and-long-term-outcome/