Pathology of Primary Angiitis of the Central Nervous System

Primary angiitis of the central nervous system (PACNS) is a rare and potentially severe form of vasculitis that is limited to the brain, spinal cord, and meninges. Despite extensive research, the etiology and underlying immunologic mechanisms of PACNS remain largely unknown. PACNS presents with a variety of clinical, radiological, and pathologic features, but it is generally characterized by inflammation and destruction of the walls of blood vessels in the CNS, which can lead to tissue ischemia and/or hemorrhage. Three main histopathologic patterns have been identified, namely granulomatous, lymphocytic, and necrotizing vasculitis.