To present a case of AAV in a young patient with multiple recurrent strokes after COVID-19 vaccination.

AAV is a relatively rare small-vessel necrotizing vasculitis. CNS involvement including ischemic strokes is usually uncommon but has been reported to be the initial disease manifestation. Typical features include multi-territory infarcts with high susceptibility for hemorrhagic transformation. New-onset AAV is a rare but known complication after COVID-19 vaccination. 

33-year-old previously healthy female who presented with multiple recurrent strokes in different vascular territories in addition to brainstem FLAIR hyperintensities and frontal SAH. She also had constitutional symptoms, headaches and joint pain. ESR 117, CRP 30. Infectious workup including pan-cultures were negative. MRA head with vessel wall imaging was concerning for vasculopathy. DSA was negative for vasculitis. EMG/NCS showed asymmetric axonal sensorimotor neuropathy. Patient was transferred to MICU for sympathetic hyperactivity/dysautonoimia and was treated with clonidine. Labs were notable for positive ANA, p-ANCA, antiphospholipid antibodies, and dsDNA. Complement, CCP, MPO, PR3, cryoglobulins were negative. LP showed Protein of 72, TNC of 32, glucose of 31 and meningitis/encephalitis panel was negative. PET scan showed multiple hypermetabolic lymph nodes. Anticoagulation was started for hypercoagulability in the context of positive antiphospholipid antibodies. In the setting of hyperinflammation, patient was started on PLEX and anakinra 200 mg BID. She also received cyclophosphamide and high dose steroids. Biopsy of Left peroneal nerve and peroneus longus muscle showed necrotizing vasculitis. Despite maximal medical management, patient continued to deteriorate until her family decided to transition to comfort cares. Patient passed away. Autopsy confirmed findings of vasculitis. Of note, the patient had COVID vaccine booster weeks before her symptoms started. 

This case highlights the importance of having a high index of suspicion in diagnosis and early treatment in young patients with autoimmune strokes who have no clear alternative etiology.