Central Nervous System Vasculitis in Rheumatoid Arthritis

Abstract

SUMMARY:The history of a patient with rheumatoid arthritis and necrotizing vasculitis affecting only the central nervous system is reported. Clinical and pathological involvement by this process was present in both cerebral hemispheres, the pons and spinal cord. Review of the literature revealed that cerebral vasculitis in rheumatoid arthritis has been reported rarely and spinal cord vasculitis not at all.

Recovery from Rheumatoid Cerebral Vasculitis by Low-Dose Methotrexate.

“…Moreover, the published observations of cases of rheumatoid cerebral vasculitis, in whomRAhad been treated with steroids, revealed a poor prognosis (2)(3)(4)(5)(6)(7)(8)(9). Steroid therapy over a long period of time might have affected cerebral vasculitis in the present case.…”

“…These manifestations result from various causes associated with the diversity of the disease process of RA (1). Cursory review of the literature revealed very rare cases of cerebral vasculitis in RA, usually with a fatal outcome (2)(3)(4)(5)(6)(7)(8)(9). Here, we describe a patient with RAwho developed cerebral infarction due to vasculitis of vertebrobasilar arteries, and showed striking clinical and angiographic recovery by low-dose methotrexate (MTX)therapy.…”

Rheumatoid Arthritis with Neurological Involvement Manifested as Hemispheric Masses

“…CNS disease occurs in a significant number of patients without active synovitis and extracranial vasculitis and nodules. CNS vasculitis in patients with RA may manifest as necrotizing vasculitis [4] or arterial plexiform change with amyloidosis [5] . These findings, however, were not observed in our patient.…”

Nonatherosclerotic Vasculopathies

“…Some reports note that neurologic defi cits occasionally persist, and occlusions of large intracranial arteries and their branches are sometimes found. 198,[244][245][246][247] In patients with active RA, levels of fi brinogen, fi brinogen turnover, and fi brin degradation products are increased. 238 Some patients with TTP develop an encephalopathy associated with headache, seizures, and visual loss, accompanied by reversible brain-imaging abnormalities predominantly located in the posterior portions of the cerebral hemispheres.…”

“…232,236,237 Brain hemorrhages have also been reported. [244][245][246][247][248] Also, high titers of circulating rheumatoid factor can cause a hyperviscosity syndrome. 238 This reversible posterior leukoencephalopathy syndrome is related to altered renal function and probably represents a "capillary leak" syndrome that is potentially reversible.…”