AutoiMMune/inflAMMAtory optic neuropAthies

InTRoDUCTIon Inflammatory, demyelinating optic neuritis is one of the most common optic neuropathies in young adults. It occurs idiopathically, or in association with multiple sclerosis, and follows a typical course of progressing visual dysfunction often with pain for one – two weeks, followed by significant resolution over the next few weeks. Due to the characteristic presentation and frequency of this presumably autoimmune process, optic neuritis is often high on the differential diagnosis for patients presenting with optic neuropathy. However, it is important to recognize that a number of other autoimmune disorders and systemic or localized inflammatory processes can also present with an optic neuropathy. While the presenting signs and symptoms often are distinct from typical inflammatory demyelinating optic neuritis, patients can present with very similar findings that may make diagnosis difficult. Here, the distinct features and etiologies of other automimmune/ inflammatory optic neuropathies (the zebras) are discussed, including neuromyelitis optic, systemic autoimmune diseases such as systemic lupus erythematosus, idiopathic orbital inflammatory syndrome with optic perineuritis, and sarcoid optic neuropathy. Potential overlapping features, as well as underlying etiologies, are examined.