The subjects of this review are the various types of optic nerve disease that involve autoimmune mechanisms, broadly defined. These include optic neuropathy caused by direct inflammatory involvement of the nerve parenchyma and optic nerve damage as an ischemic sequel to primary vasculitis, although there is naturally some overlap between the two conditions. The review considers 1) optic neuropathy that occurs as part of recognized systemic autoimmune disorders and collagen vascular diseases; and 2) isolated autoimmune optic neuropathy in which there is usually some serologic marker indicating the autoimmune nature of the process but without the signs or symptoms of a systemic disease and without the standard criteria for diagnosing a specific systemic disease.