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Central Nervous System (CNS) Vasculitis

 

Vasculitis of the CNS is an inflammation of the brain's blood vessels due to lupus activity.
The most serious of the CNS syndromes associated with lupus, it was the first to be described & is one of only two CNS syndromes (the other being psychosis) that fulfill the ACR criteria for defining lupus.

Vasculitis of the CNS usually occurs early in the disease course; over 80% of episodes take place within 5 years of diagnosis.
The typical patient experiences high fevers, seizures, meningitis-like stiffness of the neck, & may manifest psychotic or bizarre behaviour.
10% of lupus patients develop CNS vasculitis.

Untreated, their course rapidly deteriorates into stupor & ultimately coma.
 It is diagnosed either by a conventional angiogram, a magnetic resonance angiogram, lumbar puncture, or tests to detect high levels of antineuronal antibodies in the serum.

It is treated with high doses of corticosteroids, or cytotoxic drugs, & can be treated successfully.

 

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