Characterization of Extracranial Giant Cell Arteritis with Intracranial Involvement and its Rapidly Progressive
Carolin Beuker1, Maximilian Christian Wankner2, Christian Thomas3
1Department of Neurology with Institute of Translational Neurology, University of Münster, Münster, Germany.
Insights
Giant cell arteritis (GCA) with intracranial involvement often lacks typical symptoms like headache and elevated ESR. Vertebral artery stenosis patterns help differentiate GCA from atherosclerosis, with IL-6 and IL-17 as potential therapeutic targets.
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.
Objective:
The objective of this study was to characterize patients with extracranial giant cell arteritis with intracranial involvement.
Methods:
In a multicenter retrospective study, we included 31 patients with systemic giant cell arteritis (GCA) with intracranial involvement. Clinical characteristics, pattern of arterial involvement, and cytokine profiles were assessed. Patients with GCA without intracranial involvement (n = 17), and with intracranial atherosclerosis (n = 25) served as controls.
Results:
Erythrocyte sedimentation rate (ESR) was elevated in 18 patients (69.2%) with and in 16 patients (100%) without intracranial involvement (p = 0.02). Headache was complained by 15 patients (50.0%) with and 13 patients (76.5%) without intracranial involvement (p = 0.03). Posterior circulation arteries were affected in 26 patients (83.9%), anterior circulation arteries in 17 patients (54.8%), and both territories in 12 patients (38.7%). Patients with GCA had vertebral artery stenosis proximal and, in contrast, patients with atherosclerosis distal to the origin of posterior inferior cerebellar artery (PICA). Among patients with GCA with intracranial involvement, 11 patients (37.9%) had a rapid progressive disease course characterized by short-term recurrent ischemic events. The median modified Rankin Scale (mRS) at follow-up in these patients was 4 (interquartile range [IQR] = 2.0-6.0) and 4 patients (36.4%) died. Vessel wall expression of IL-6 and IL-17 was significantly increased in patients with rapid progressive course.
Interpretation:
Typical characteristics of GCA, headache, and an elevated ESR, are frequently absent in patients with intracranial involvement. However, differentiation of intracranial GCA from atherosclerosis can be facilitated by the typical pattern of vertebral artery stenosis. About one-third of patients with intracranial GCA had a rapid progressive course with poor outcome. IL-17 and IL-6 may represent potential future treatment targets. ANN NEUROL 2021;90:118-129.
