Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): Clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis

Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major c auses of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be consid ered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE co uld be considered to be a 'general' neurological disease due to different i mmunological mechanisms, such as vasculitis. Additionally, the literature o n SLE patients affected by optic neuropathy is reviewed to evaluate the maj or clinical features, particularly neurological features. In reviewing the Literature, it appears that bilateral optic neuropathy in SLE occurs more f requently than monolateral optic neuropathy, and the main neurological mani festation seen in these patients is transverse myelitis, particularly in SL E patients with bilateral optic nerve disease. Finally, we propose a clinic o-ophthalmological spectrum of APS and outline the ocular clinical manifest ations that can be considered as diagnostic for the syndrome.