Optic Neuropathy in Systemic Lupus Erythematosus and Antiphospholipid Syndrome (APS): Clinical Features, Pathogenesis, Review of the Literature and Proposed Ophthalmological Criteria for APS Diagnosis

Abstract

Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes 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 considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.

Ocular involvement in connective tissue disorders

“…It is thought to be attributed to small vessel ischemia [32,33]. Ischemic optic neuropathy may also complicate polyarteritis nodosa and Wegener's granulomatosis.…”

“…Optic neuropathy, which may be anterior or posterior, is a well-known ocular manifestation occurring in patients with SLE [32,33]. It is thought to be attributed to small vessel ischemia [32,33].…”

Ocular Manifestations in Systemic Lupus Erythematosus

“…Retinal vasculitis, vitiritis, posterior scleritis and central retinal artery occlusion also reported with higher prevelance in aPL positive patients [110][111][112][113]. APS associated neuro-ophthalmic changes are probably related to blood supply hazard and optic nerve demyelization and include anterior non-arterial ischemic neuropathy (NAION), retro-bulbar neuritis and orbital ischemic syndrome on catastrophic APS [114,115].…”

Sudden Onset Blindness in a Patient with Mixed Connective Tissue Disease

“…2,3 Lupus retinopathy is one of the most common vision-threatening complications of SLE, occurring in up to 29% of patients. [6][7][8] Another etiology to consider is rheumatoid arthritis (RA). Blood vessel inflammation is a central feature of RA.…”