Autoimmune optic neuropathy
A rare optic neuropathy with autoimmune serology and poor visual prognosis.
Last updated
Autoimmune optic neuropathy (AON) is a clinical syndrome characterized by chronically progressive or recurrent vision loss associated with serological evidence of autoimmunity, particularly positive antinuclear antibody (ANA) despite not meeting criteria for systemic lupus erythematosus (SLE). It was first described in 1982 and is considered a possible forme fruste of SLE-associated optic neuropathy.
Quick Facts
- First described
- 1982
- Associated condition
- Systemic lupus erythematosus (SLE)
- Key feature
- Steroid-responsive and steroid-dependent vision loss
- Prevalence in sle
- 1-2% of SLE patients develop optic neuropathy
Facts from the source article.
Lore & Background
Autoimmune optic neuropathy (AON) was first described in 1982. It presents with visual loss and signs of optic nerve dysfunction, such as loss of color vision, afferent pupil defect, and sometimes abnormalities of the optic disc.
The clinical features can be variable and include acute anterior or retrobulbar optic neuritis sometimes associated with pain, anterior or retrobulbar ischemic optic neuropathy not associated with pain, and chronic progressive vision loss that mimics a compressive lesion. The main features that differentiate AON from typical demyelinating optic neuritis are poor recovery of vision and a chronic, recurrent, or bilateral course. Workup for multiple sclerosis, including MRI, will be negative.
Reader's Guide
Autoimmune optic neuropathy represents a distinct clinical entity separate from typical demyelinating optic neuritis and from lupus optic neuritis in patients with defined SLE. Its significance lies in its poor visual prognosis and its requirement for chronic immunosuppression, unlike typical optic neuritis. The pathogenesis is hypothesized to involve inflammation from auto-antibodies, immune complexes, T-cells, and complement, damaging optic nerve components and blood vessels, leading to vasculitis and ischemia. The poor recovery despite anti-inflammatory treatment suggests ischemia is an important component.
AON is rare, and its natural history is not well defined. Early diagnosis and prompt treatment with systemic corticosteroids may restore some visual function, but patients often remain steroid dependent, and vision worsens when corticosteroids are tapered. Long-term steroid-sparing immunosuppressive agents may be required to limit side-effects and minimize risk of worsening vision. No formal recommendation can be made regarding the best therapeutic approach.
More in Steroid-responsive inflammatory conditions
Sources
Compiled from Wikipedia and the sources listed below. Text from Wikipedia is available under CC BY-SA 4.0; this entry is adapted from it.
- Wikipedia: Autoimmune optic neuropathy (CC BY-SA 4.0).
- Word definitions: the Codexery glossary, each quoted from its Wikipedia article.
Spotted an error? Know more?
Reader corrections go straight into our review queue. Suggest an edit · How this site is sourced