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Granulomatous angiitis, associated with Hashimoto’s thyroiditis and anticardiolipin antibodies, has been identified as the fourth primary etiology of idiopathic CNS vasculitis. Central nervous system vasculitis can be a devastating disorder which typically occurs in the third and fourth decades of life, and is associated with a poor prognosis. Establishing the diagnosis of idiopathic CNS vasculitis usually requires a combination of biopsy findings and a constellation of inflammatory findings in the blood and cerebrospinal fluid. The pathogenesis of CNS vasculitis is not known, but immunological mechanisms are suspected. Serum and CSF autoantibodies are often elevated in patients with CNS vasculitis, but have not been useful for diagnostic purposes. We measured DNA autoantibodies and found that four of nine patients with documented CNS vasculitis had high levels of DNA autoantibodies. Three patients had Hashimoto’s thyroiditis, two patients had anti-beta 2 glycoprotein antibodies, and one patient had antimitochondrial antibodies. These findings support the hypothesis that DNA autoantibodies may be important in the pathogenesis of idiopathic CNS vasculitis. Further studies are required to determine whether DNA autoantibodies can be used as a diagnostic marker and to delineate their pathogenetic role./*
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