Objective:

NA

Background:

Rheumatoid central nervous system (CNS) vasculitis is an uncommon, but potentially life-threatening extra-articular complication of rheumatoid arthritis (RA). The spectrum of symptoms varies with the vessel involved, often complicating timely diagnosis and management.

Design/Methods:

NA

Results:

A 52-year-old man with a history of erosive seropositive RA, migraine, and type 2 diabetes presented with two days of right temporal throbbing headache with associated nausea, vomiting, photophobia, phonophobia, and blurred vision. Although his presentation resembled his usual migraines, his standard rescue therapy failed to abort the headache, prompting him to seek further care. Serologic workup demonstrated an elevated erythrocyte sedimentation rate of 93 mm/hr and C-reactive protein of 198.8 mg/L. Antineutrophil cytoplasmic antibody was negative. Brain magnetic resonance imaging (MRI) with gadolinium contrast was unremarkable. He developed scalp tenderness on day 4 of admission, without jaw claudication. Bilateral temporal artery biopsy was negative for giant cell arteritis. Lumbar puncture on day 10 of admission was consistent with an inflammatory process with elevated CSF protein at 158 mg/dL, lymphocytic-predominant pleocytosis (WBC 17, 80% lymphocytes), and negative infectious panels. Finally, an MR vessel wall imaging (VW-MRI) and time-of-flight MR angiography (MRA) revealed delayed concentric wall thickening and enhancement of the bilateral V4 segments, suggestive of vasculitis. A diagnosis of CNS vasculitis was established, and the patient was treated with a 3-day course of intravenous methylprednisolone (1,000 mg daily) followed by a steroid taper and initiation of rituximab (1 g every two weeks), resulting in complete resolution of symptoms.

Conclusions:

Rheumatoid CNS vasculitis is a rare manifestation of RA with heterogeneous clinical presentations. Advanced imaging techniques, such as MR vessel wall imaging, may facilitate earlier diagnosis and treatment of this potentially fatal condition and should be considered more frequently during evaluation.