What is the treatment for ocular sarcoidosis?

Treatment of Ocular Sarcoidosis

Topical corticosteroids are the first-line treatment for ocular sarcoidosis, with systemic corticosteroids indicated for cases with posterior uveitis, bilateral involvement, or inadequate response to topical therapy. When ocular sarcoidosis does not respond to corticosteroids or requires unacceptable doses to maintain remission, immunosuppressive agents (particularly methotrexate) should be added as steroid-sparing therapy 1, 2.

Treatment Algorithm for Ocular Sarcoidosis

First-Line Treatment

• Anterior Uveitis/External Ocular Involvement

  • Topical corticosteroid drops (prednisolone acetate 1% or dexamethasone 0.1%)
  • Initial frequency: Every 1-2 hours, tapering based on response
  • Cycloplegic agents (e.g., cyclopentolate) to prevent synechiae formation and relieve pain
  • Artificial tears for associated dry eye symptoms 1, 2

• Posterior Uveitis/Bilateral Involvement/Optic Neuropathy

  • Oral prednisone 20-40 mg daily for 3-6 months with gradual taper 3, 4
  • Higher doses (1 mg/kg/day) may be required for optic neuropathy 2
  • Target: Lowest effective dose to control inflammation

Second-Line/Steroid-Sparing Treatment

For patients requiring prolonged corticosteroid therapy (>3 months), unacceptable side effects, or inadequate response:

• Methotrexate: 10-15 mg once weekly (most commonly used) 3, 2, 5

  • Monitor: CBC, liver function tests
  • Supplement with folic acid to reduce side effects

• Alternative immunosuppressants if methotrexate is contraindicated or ineffective:

  • Azathioprine: 50-250 mg daily
  • Mycophenolate mofetil: 500-1500 mg twice daily
  • Leflunomide: 10-20 mg daily 3, 5

Third-Line/Refractory Disease Treatment

For cases not responding to conventional immunosuppressants:

• Biologic agents:
  • Infliximab: 3-5 mg/kg initially, then at 2 weeks, followed by every 4-6 weeks 3, 6, 7
  • Adalimumab: 40 mg every 1-2 weeks 1, 3, 7

Local Therapy for Specific Complications

• Cystoid macular edema: Intravitreal corticosteroid implants 2, 7
• Secondary glaucoma: Topical IOP-lowering medications
• Dry eye disease: Artificial tears containing methylcellulose or hyaluronate at least twice daily 1

Monitoring and Follow-up

• Regular ophthalmologic examinations with slit lamp biomicroscopy
• Intraocular pressure monitoring
• Optical coherence tomography for macular edema
• Fluorescein and indocyanine green angiography to detect chorioretinal granulomas 8
• Monitor for medication side effects:
  • Corticosteroids: Blood pressure, blood glucose, bone density
  • Methotrexate: CBC, liver function tests
  • Biologics: Screen for latent tuberculosis before initiation

Special Considerations

• Optic neuropathy: Often affects women of African and Caribbean descent and may require more aggressive treatment with high-dose corticosteroids plus concurrent immunosuppression from disease onset 2
• Elderly Caucasian women: Often present with chronic posterior uveitis requiring systemic therapy 2
• Pneumocystis prophylaxis: Consider in patients on high-dose immunosuppression (≥20 mg prednisone with cytotoxic agent for >6 months) 3

Treatment Pitfalls to Avoid

• Delaying systemic therapy for bilateral posterior uveitis or optic neuropathy
• Inadequate monitoring for corticosteroid-induced complications
• Failing to recognize treatment-resistant cases that require early escalation to immunosuppressive therapy
• Not considering differential diagnoses (tuberculosis, syphilis) when treatment response is poor 8

The European Respiratory Society notes that while eye involvement occurs in a significant number of sarcoidosis cases, there are few studies specifically regarding treatment of ocular sarcoidosis, making treatment decisions challenging 1. However, the available evidence supports a stepwise approach starting with topical therapy for anterior disease and progressing to systemic medications for more severe or posterior involvement.