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Systemic lupus erythematosus and antineutrophilic cytoplasmic antibody-associated vasculitis overlap syndrome complicated by subarachnoid hemorrhage: case-based review

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Khorolsky C1, Castellano A2, Comstock D2, Brinster NK3, See SY4, Garner BF5. Rheumatol Int. 2018 Dec;38(12):2329-2335. doi: 10.1007/s00296-018-4169-z. Epub 2018 Oct 16.



Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) overlap syndrome is an inflammatory disorder with a mixed presentation that is characterized by clinical features of both SLE and AAV. Although renal disease predominates, any organ system in the body may be affected. Neurologic manifestation in patients with SLE-AAV overlap syndrome is rare and has only been previously documented as cerebral ischemia. We report a patient with SLE-AAV overlap syndrome diagnosed based on clinical, serologic and biopsy-proven histologic findings who presented with subarachnoid hemorrhage (SAH) secondary to ruptured right anterior cerebral artery aneurysm. To the authors' knowledge, this is the first reported case of SLE-AAV overlap syndrome diagnosed in a patient with a SAH due to an intracranial aneurysm. Neurologic involvement in patients with SLE-AAV overlap syndrome is uncommon and has not been well-studied. Clinicians who encounter patients with neurologic signs that present with symptoms and a serologic profile that correspond to both SLE and AAV criteria, should consider the association between SLE-AAV overlap syndrome and a hemorrhagic stroke, specifically SAH.