A Case of Rheumatoid Cerebral Vasculitis Presenting to the ED

Abstract

Central nervous system involvement in rheumatoid arthritis is infrequent. The most frequent neurological manifestations of rheumatoid arthritis are peripheral neuropathy and cervical spinal cord compression due to subluxation of the cervical vertebrae. Cerebral rheumatoid vasculitis is an uncommon and serious complication which can be life-threatening Neurological involvement in RA is rare, present in only 1% of patients. Disorders of the central nervous system (CNS) include cervical myelopathy, vasculitis, RNs located within the CNS, or meningitis. Stroke also occurs with increased frequency. CNS vasculitis is extremely rare. The diagnosis is supported by magnetic resonance imaging (MRI), alone or with magnetic resonance angiography (MRA), showing the segmental vascular stenosis characteristic of vasculitis. Peripheral neuropathy is usually manifested as sensorimotor neuropathy or mononeuritis multiplex. The underlying mechanism is small vessel vasculitis of the vasa vasorum of the nerves with ischaemic neuropathy and demyelinisation as part of the rheumatoid vasculitis (RV) syndrome. Rheumatoid vasculitis typically affects small and medium-size blood vessels. It is associated with high rates of premature mortality with up to 40% of patients dying by 5 years as well as significant morbidity due to both organ damage from vasculitis and consequences of the treatment. High levels of circulating immune complexes have been observed in atients with rheumatoid vasculitis, and in particular high serum levels of rheumatoid factor are often detected at the time of onset of vasculitis.Deposition of immune complexes most likely contributes to small vessel inflammation and organ damage. Anti CCP levels also tend to be higher in patients with RA who have systemic vasculitis than in those who do not. Histologically, rheumatoid vasculitis involves blood vessels of the small arteries, and all layers of the vessel wall are infiltrated by neutrophils, lymphocytes, and plasma cells.