Walsh & Hoyt: Vasculitides

Two main categories of vasculitis may produce neuro-ophthalmologic manifestations: (a) primary central nervous system (CNS) vasculitides, and (b) systemic necrotizing vasculitides that often affect the CNS. Strict classification of vasculitis is difficult because the etiology and pathogenesis of many vasculitic entities remain unknown. The main disorders that compose the primary CNS vasculitides are granulomatous angiitis of the nervous system (GANS), Cogans syndrome, and acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The disorders that compose the group of systemic necrotizing vasculitides that often affect the CNS are polyarteritis nodosa (PAN), Churg-Strauss disease, giant-cell arteritis (GCA), Takayasus arteritis (pulseless disease), Wegeners granulomatosis, Henoch-Schonlein purpura, and mixed cryoglobulinemia. In the following sections, we discuss each of these disorders as they pertain to neuroophthalmology.