TY - JOUR
T1 - Clinical and Radiographic Features of Giant Cell Arteritis With Intracranial Involvement
AU - Sanchez-Alvarez, Catalina
AU - Hawkins, Alexander S.
AU - Koster, Matthew J.
AU - Lehman, Vance T.
AU - Crowson, Cynthia S.
AU - Warrington, Kenneth J.
N1 - Publisher Copyright:
© 2020 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.
PY - 2020/8/1
Y1 - 2020/8/1
N2 - Objective: Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects the aorta and its branches. Extracranial branches of the carotid artery are frequently affected; however, intracranial involvement in GCA is rare. Methods: A retrospective medical record review was performed to identify all patients with intracranial GCA (IC-GCA) from January 1996 through May 2018. Results: Nine patients with IC-GCA were included (78% male; mean age, 72.1 years [SD: 7.9]). All patients met ACR criteria for GCA. The median time from onset of GCA to intracranial involvement was 0.6 months (interquartile range: 0.1-5.1). All patients had neurologic symptoms, 89% had an ischemic cerebrovascular event. Transient or permanent vision loss was frequent (56% of patients). IC-GCA was diagnosed by cranial imaging in all nine patients. Intracranial vasculitis most commonly affected the internal carotid artery (100%), followed by the vertebral artery (67%), posterior cerebral artery (67%), middle cerebral artery (44%), anterior cerebral artery (33%), and posterior inferior cerebral artery (11%). Intracranial vessel stenosis was present in 89%, occlusion in 33%, wall thickening in 33%, and dilation in 11%. All patients received glucocorticoids. Additional therapeutic agents included cyclophosphamide (67%) and tocilizumab (22%). Despite treatment, five patients had rapid deterioration and mortality. Comparing IC-GCA patient survival to the expected rates from the US population, the standardized mortality ratio (95% CI) for IC-GCA was 58.1 (18.9-135.6). Conclusion: Although rare, IC-GCA is associated with significant morbidity and mortality. It occurs predominantly in men and presents with ischemic cerebrovascular events. Current treatment strategies appear to be of limited efficacy for IC-GCA.
AB - Objective: Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects the aorta and its branches. Extracranial branches of the carotid artery are frequently affected; however, intracranial involvement in GCA is rare. Methods: A retrospective medical record review was performed to identify all patients with intracranial GCA (IC-GCA) from January 1996 through May 2018. Results: Nine patients with IC-GCA were included (78% male; mean age, 72.1 years [SD: 7.9]). All patients met ACR criteria for GCA. The median time from onset of GCA to intracranial involvement was 0.6 months (interquartile range: 0.1-5.1). All patients had neurologic symptoms, 89% had an ischemic cerebrovascular event. Transient or permanent vision loss was frequent (56% of patients). IC-GCA was diagnosed by cranial imaging in all nine patients. Intracranial vasculitis most commonly affected the internal carotid artery (100%), followed by the vertebral artery (67%), posterior cerebral artery (67%), middle cerebral artery (44%), anterior cerebral artery (33%), and posterior inferior cerebral artery (11%). Intracranial vessel stenosis was present in 89%, occlusion in 33%, wall thickening in 33%, and dilation in 11%. All patients received glucocorticoids. Additional therapeutic agents included cyclophosphamide (67%) and tocilizumab (22%). Despite treatment, five patients had rapid deterioration and mortality. Comparing IC-GCA patient survival to the expected rates from the US population, the standardized mortality ratio (95% CI) for IC-GCA was 58.1 (18.9-135.6). Conclusion: Although rare, IC-GCA is associated with significant morbidity and mortality. It occurs predominantly in men and presents with ischemic cerebrovascular events. Current treatment strategies appear to be of limited efficacy for IC-GCA.
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U2 - 10.1002/acr2.11161
DO - 10.1002/acr2.11161
M3 - Article
AN - SCOPUS:85103914918
SN - 2578-5745
VL - 2
SP - 471
EP - 477
JO - ACR Open Rheumatology
JF - ACR Open Rheumatology
IS - 8
ER -