Characterization of Extracranial Giant Cell Arteritis with Intracranial Involvement and its Rapidly Progressive...

Area of Science:

• Neurology
• Vascular Inflammation
• Rheumatology

Background:

• Giant cell arteritis (GCA) is a large-vessel vasculitis primarily affecting extracranial arteries.
• Intracranial involvement in GCA is less common but can lead to severe neurological complications.
• Distinguishing intracranial GCA from atherosclerosis is crucial for appropriate management.

Purpose of the Study:

• To characterize the clinical features, arterial involvement patterns, and cytokine profiles of patients with GCA and intracranial disease.
• To compare these features with patients with GCA without intracranial involvement and those with intracranial atherosclerosis.

Main Methods:

• Multicenter retrospective study including 31 patients with systemic GCA and intracranial involvement.
• Assessment of clinical characteristics, arterial involvement patterns, and cytokine profiles.
• Comparison with control groups: GCA without intracranial involvement (n=17) and intracranial atherosclerosis (n=25).

Main Results:

• Elevated ESR was less frequent in patients with intracranial GCA (69.2%) compared to those without (100%).
• Headache was also less common in the intracranial GCA group (50.0%) versus the control group (76.5%).
• Posterior circulation arteries were predominantly affected in intracranial GCA, with a distinct pattern of vertebral artery stenosis compared to atherosclerosis. A rapid progressive course was observed in 37.9% of patients, associated with increased IL-6 and IL-17 expression.

Conclusions:

• Typical GCA symptoms like headache and elevated ESR may be absent in cases with intracranial involvement.
• Vertebral artery stenosis patterns can aid in differentiating intracranial GCA from atherosclerosis.
• Intracranial GCA can have a poor prognosis, and IL-6 and IL-17 may be future therapeutic targets.