Abstract Details

Title A Novel Overlap of Hypocomplementemic Urticarial Vasculitis and Moyamoya Syndrome

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P1 - Poster Session 1 (8:00 AM-9:00 AM)

Poster/Presentation
Number 4-017

Objective To describe a rare overlap between systemic hypocomplementemic urticarial vasculitis and moyamoya-pattern intracranial vasculopathy presenting as ischemic stroke, and to propose a unifying immunovascular mechanism.

Background Moyamoya syndrome is an uncommon cerebrovascular disorder associated with specific systemic diseases. Its coexistence with hypocomplementemic urticarial vasculitis has not been reported. Central nervous system involvement in this vasculitis typically affects small vessels rather than producing large-artery stenosis.

Design/Methods A 26-year-old male with biopsy-proven leukocytoclastic vasculitis, chronic hypocomplementemia, and recurrent systemic inflammatory flares presented with acute left arm weakness and headache. Brain and vascular imaging demonstrated multifocal right MCA infarcts with severe M1 stenosis. Digital subtraction angiography revealed moyamoya-type (“puff-of-smoke”) collateralization. Comprehensive evaluation excluded cardioembolic, atherosclerotic, and infectious etiologies. He was managed with immunomodulatory and cerebrovascular therapies.

Results This case of biopsy-confirmed systemic vasculitis with moyamoya-type vascular remodeling supports a model of immune-augmented moyamoya, in which systemic immune dysregulation accelerates intracranial arteriopathy and collateral formation. Continued ischemic progression despite immunotherapy suggests a fixed arteriopathy.

Conclusions Systemic immune activation may promote moyamoya-pattern arterial remodeling in susceptible individuals. Recognizing this overlap is essential for integrating immunologic and revascularization approaches in young stroke patients presenting with combined inflammatory and cerebrovascular disease.

Authors/Disclosures
Haidar Haidar PRESENTER	Mr. Haidar has nothing to disclose.
Amina Benguedouar	Ms. Benguedouar has nothing to disclose.
Hadeel Shalabi, MD	Dr. Shalabi has nothing to disclose.
Daniel Miller, MD	Dr. Miller has nothing to disclose.

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