Autoimmune optic neuropathy
Steroid-responsive optic neuropathy with serological autoimmunity.
Autoimmune optic neuropathy (AON) is a clinical syndrome characterized by chronically progressive or recurrent vision loss with serological evidence of autoimmunity, often positive ANA, but without meeting criteria for systemic lupus erythematosus. It may represent a forme fruste of SLE-associated optic neuropathy. The condition is notable for being steroid-responsive yet steroid-dependent, with a worse visual prognosis than typical demyelinating optic neuritis.
Quick Facts
- First described
- 1982
Facts from the source article.
Signs and symptoms
AON presents with visual loss and signs of optic nerve dysfunction, including loss of color vision, afferent pupil defect, and sometimes optic disc abnormalities. Clinical forms include acute anterior or retrobulbar optic neuritis, sometimes painful; anterior or retrobulbar ischemic optic neuropathy without pain; and chronic progressive vision loss mimicking a compressive lesion. Differentiating features from typical demyelinating optic neuritis are poor visual recovery, chronic, recurrent, or bilateral course, and negative MRI for multiple sclerosis.
Pathogenesis
About 1-2% of patients with defined SLE develop optic neuropathy, rarely as the presenting sign. The hypothesized pathogenesis involves inflammation from auto-antibodies, immune complexes, T-cells, and complement damaging optic nerve components and blood vessels, causing vasculitis and ischemia. This combination may produce reversible demyelination or permanent axonal necrosis. Poor recovery despite anti-inflammatory treatment suggests ischemia from vasculitis is important; AON may represent an incomplete expression of SLE-associated optic neuropathy.
Treatment
AON is rare with an undefined natural history. Unlike typical optic neuritis, it is not associated with multiple sclerosis and has a worse visual prognosis. Treatment often involves chronic immunosuppression. Early systemic corticosteroids may restore some vision, but patients often remain steroid-dependent, with vision worsening on taper. Long-term steroid-sparing immunosuppressive agents may be needed to limit steroid side effects and minimize risk of vision loss.
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