Stroke in the Young: A Case of CNS Vasculitis Secondary to Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an enigmatic autoimmune disease with a varied presentation that carries with it an increased risk for many potentially life-threatening problems. When compared with the general population, the relative risk of ischemic stroke in SLE is more than doubled. Our case report highlights a rare complication of SLE, vasculitis as the cause of stroke. CNS vasculitis secondary to SLE most commonly affects the endothelium of small and medium vessel arteries through perivascular inflammation in a concentric pattern. We present a case of a 46-year-old female with a history of hypertension, hyperlipidemia, hypothyroidism, multiple ischemic strokes (residual left sided weakness), and elevated cardiolipin IgM levels (not on anticoagulation) who presented with left facial numbness, dysarthria, and word finding difficulty. The initial CT head revealed chronic appearing hypodensities in the right parietal and occipital lobes without hemorrhage. CT angiogram of the head and neck demonstrated multifocal stenosis in the right MCA and right PCA. She received IV tenecteplase and MRI brain revealed an infarct in the left MCA distribution along with infarcts in the right thalamus, hippocampus, and cerebellum. Transesophageal echocardiogram was negative for Libman-Sacks endocarditis. Given concerns for an underlying vasculitis process, an MRA vessel wall imaging was obtained which demonstrated concentric enhancement and persistent vessel narrowing in the left MCA M2 along with symmetric concentric enhancement of vaso vasorum of intracranial vertebral arteries. Lumbar puncture revealed CSF protein 60, culture negative, glucose 61, white blood cells 3, IgG index 0.5, ACE negative, and no oligoclonal bands. Her ESR was 43 and CRP < 0.5. Hypercoagulability workup demonstrated RNP > 8, SSA 6.4, Smith 1.6, +ANA 1:1280, C4 11, cardiolipin IgM 60. Given serum ANA titers and anti-cardiolipin IgM labs in the setting of intracranial vessel wall enhancement concerning for an inflammatory process, it was believed that this stroke was actually a result of lupus vasculitis. The paucity of literature on the association between SLE vasculitis and cerebrovascular disease presents an important opportunity for more research, and it may be prudent to further investigate the relative risk of stroke in patients with known SLE vasculitis. This is also important because young individuals with SLE are at a higher relative risk of stroke.