Primary angiitis

Primary angiitis of the CNS causes inflammation particularly in small leptomeningeal vessels, whereas larger vessels can also be affected. Systemic variants of vasculitis and secondary arteritis of the CNS affect mostly small or medium-sized arteries to different degrees, and occasionally the venous system is also afflicted as in Behcet disease. Segmental stenoses are frequently found, often not including bifurcations and alternating with arterial dilatations. This pattern is not definitively specific and can also be seen in atherosclerosis. Even with an optimal MRA technique, DSA still remains necessary for the depiction of tiny vessel lesions (Fig. 5.26). 

Inflammatory conditions Primary angiitis Systemic lupus erythematosus Temporal arteritis Takayasu s arteritis... 

Bimbaum J, Hellmann DB Primary angiitis of the central nervous system. Arch Neurol 2009 66 pp. 704-709. 

A 50-year-old woman was given interferon alfa for chronic hepatitis C and primary biliary cirrhosis, and within 2 months became febrile and developed a diffuse nodular erythematous rash. The skin biopsy showed typical features of necrotizing angiitis, and cutaneous periarteritis nodosa was diagnosed. Full recovery was obtained after interferon alfa withdrawal and prednisolone treatment. 

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