Treatment of Autoimmune Disorders Affecting the Eye
For autoimmune eye disease, initiate systemic corticosteroids combined with steroid-sparing immunosuppressive agents, with the specific regimen determined by the anatomical location and severity of ocular involvement.
Initial Assessment and Referral
All patients with suspected autoimmune eye disease require prompt ophthalmologic evaluation, particularly when presenting with:
- Moderate to severe eye pain 1
- Vision loss or visual changes 1
- Corneal infiltration or ulceration 1
- Lack of response to initial therapy 1
Ophthalmologists should manage these cases due to the broad medical skills required to diagnose and treat conditions associated with systemic immunological disorders and systemic medications 1.
Disease-Specific Treatment Algorithms
Posterior Segment Inflammatory Disease (Uveitis)
For any patient with inflammatory eye disease affecting the posterior segment, start azathioprine 2.5 mg/kg/day plus systemic corticosteroids immediately 1. This combination:
- Decreases hypopyon uveitis attacks (NNT = 4) 1
- Stabilizes visual acuity 1
- Prevents development of new eye disease (NNT = 2) 1
- Provides sustained benefit over 7-year follow-up 1
For severe posterior segment disease (defined as >2 lines drop in visual acuity on 10/10 scale and/or retinal vasculitis or macular involvement), escalate to one of the following in combination with azathioprine and corticosteroids 1:
- Ciclosporine A 2-5 mg/kg/day (first choice for rapid effect) 1
- Infliximab (particularly effective for refractory disease when combined with other immunosuppressives, though relapses common with monotherapy) 1
- Interferon-α with or without corticosteroids (second-line option) 1
Critical monitoring for ciclosporine A: Watch for hypertension and nephrotoxicity, which are the most important adverse events 1.
Critical precaution for infliximab: Screen for tuberculosis before initiation, as endemic areas for Behçet disease overlap with tuberculosis-endemic regions 1.
Anterior Segment Disease (Episcleritis)
Episcleritis typically responds to topical steroids or NSAIDs and may not require systemic treatment 1. However, treat the underlying systemic autoimmune condition concurrently 1.
Autoimmune Retinopathy
For probable autoimmune retinopathy (progressive disease within 6 months, minimal inflammation, outer retinal disruption on OCT, characteristic fundus autofluorescence abnormalities, reduced rod and cone responses on ERG, and positive antiretinal antibodies), consider immunosuppressive therapy 2.
Features making autoimmune retinopathy unlikely and requiring alternative diagnosis consideration include 2:
- Slowly progressive symptoms over years
- Bone spicules or retinal vascular sheathing
1+ anterior chamber or vitreous cells
- OCT changes predominantly at RPE level
- Diffuse retinal vasculitis on fluorescein angiography
- Normal full-field ERG
Systemic Disease Management
When systemic autoimmune disease is identified (Sjögren syndrome, rheumatoid arthritis, systemic lupus erythematosus, or other connective tissue diseases), coordinate care with rheumatology or internal medicine 1, 3.
Patients requiring long-term immunosuppressive therapy should be co-managed with internists or rheumatologists experienced in these medications 1.
Common Pitfalls and Caveats
Do not delay treatment for mild dry eye symptoms, as these may represent early manifestations of more serious autoimmune conditions like Sjögren syndrome 1.
Avoid corticosteroid monotherapy for chronic management due to risks of cataracts and glaucoma 1. Always plan for steroid-sparing immunosuppression.
Screen for tuberculosis before initiating anti-TNF therapy (infliximab, adalimumab) in all patients, as these medications increase infection risk 1, 4.
Monitor for hydroxychloroquine, chloroquine, corticosteroid, and bisphosphonate toxicity in patients with systemic autoimmune diseases, as these medications can cause iatrogenic eye injury 3.
Educate patients about the chronic nature of autoimmune eye disease and establish realistic expectations for disease control rather than cure 1.