A Novel Overlap of Hypocomplementemic Urticarial Vasculitis and Moyamoya Syndrome
Amina Benguedouar1, Haidar Haidar1, Hadeel Shalabi1, Daniel Miller1
1Henry Ford Hospital
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.
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