What is Primary Angiitis of the Central Nervous System and who gets it?
Spectrum of diseases that result from inflammation and destruction of arteries and veins of the brain, spinal cord, and meninges. The vessels become narrowed or occluded resulting in ischemia and/or necrosis in the areas of the involved vessels. PACNS is rare. Men are affected more than women (2:1). Median age of onset is 50 years, but PACNS can affect any age. Note that in the 2012 International Chapel Hill Consensus Conference, PACNS has been reclassified as a single-organ vasculitis (instead of medium-vessel vasculitis) and renamed primary CNS vasculitis (PCNSV).
