Optic neuropathy, also described as "autoimmune optic neuropathy", cna be treated successfully. It can be isolated, an early sign of or associated with systemic inflammatory disease and vasculitis. Recognition of this entity appears important since the natural course of the disease tends to end up with severe visual loss and optic atrophy. Early systemic high-dose steroid treatment and immunosuppression usually restore and maintain visual function. The clinical spectrum of this disease entity is described on the basis of the literature and our own cases, some of which have been followed up for several years. The differential diagnostic and therapeutic aspects are discussed. Association of this ill-known optic neuropathy with various inflammatory diseases is described; a rich reference list indicates its existence.