%0 Case Reports
%T Ischemic stroke or transient ischemic attack due to cervical and intracranial large-vessel vasculitis secondary to primary Sjögren's syndrome: A case series and literature review.
%A Zhu J
%A Dong Q
%A Cao Z
%A Xu Y
%A Zhang S
%A Fang Q
%A Cai X
%A Han X
%A Yang S
%J Heliyon
%V 10
%N 14
%D 2024 Jul 30
%M 39108913
%F 3.776
%R 10.1016/j.heliyon.2024.e34225
%X <B>UNASSIGNED: </B>Primary Sjögren's syndrome (PSS) is a systemic autoimmune disease that mainly affects exocrine glands. Little is known about PSS associated cervical and intracranial cerebral large-vessel vasculitis outside of individual case reports.<BR><B>UNASSIGNED: </B>We present 5 cases of ischemic stroke or transient ischemic stroke (TIA) caused by PSS associated cervical and intracranial large-vessel vasculitis. Literature review was performed to summarize and identify the demographic, clinical features, treatment, and prognosis of this condition.<BR><B>UNASSIGNED: </B>The review resulted in 8 included articles with 8 patients, plus our 5 new patients, leading to a total of 13 subjects included in the analysis. The median age was 43 (range, 17-69) years old, among which 69.2 % (9/13) were female, and 92.3 % (12/13) came from Asia. Among them, 84.6 % (11/13) presented with cerebral infarction and 70.0 % (7/10) with watershed infarction. Middle cerebral artery (MCA) (6/13, 46.2 %) and internal carotid artery (ICA) (6/13, 46.2 %) were the most frequently involved arteries. Remarkable vessel wall concentric thickening and enhancement was observed in 57.1 % (4/7) patients and intravascular thrombi was identified in 28.6 % (2/7) patients. Glucocorticoid combined with non-glucocorticoid immunosuppressants (8/12, 66.7 %) were the most often chosen medication therapy and 4 patients received surgical intervention.<BR><B>UNASSIGNED: </B>Asian females are the most vulnerable population to ischemic stroke or TIA due to PSS associated cervical and intracranial large-vessel vasculitis. Cerebral infarctions were characterized by recurrence and watershed pattern. Magnetic resonance vessel wall imaging (MR-VWI) helps to identify the inflammatory pathology of large vessel lesion in PSS.