What is the appropriate treatment and management for a 40-60 year old adult patient with scleritis, possibly having a history of autoimmune disease?

Management of Scleritis in Adults

For a 40-60 year old adult with scleritis, particularly with autoimmune disease history, initiate oral NSAIDs as first-line therapy, but add oral corticosteroids (prednisone) immediately if NSAIDs fail after 1-2 weeks or if disease is moderate-to-severe at presentation; necrotizing or posterior scleritis requires immediate systemic corticosteroids plus immunomodulatory therapy. 1

Immediate Diagnostic Priorities

• Urgent ophthalmology referral is mandatory to confirm diagnosis, classify subtype (diffuse anterior, nodular anterior, necrotizing, or posterior), and assess for vision-threatening complications 1, 2
• Distinguish scleritis from episcleritis: severe deep ocular pain, "cloudy" or discolored scleral appearance, and visual disturbances indicate scleritis, whereas episcleritis presents with superficial hyperemia and minimal pain 1, 3
• Screen for systemic autoimmune associations, particularly rheumatoid arthritis (most common), ANCA-associated vasculitis (especially granulomatosis with polyangiitis), inflammatory bowel disease, systemic lupus erythematosus, and IgG4-related disease 2, 4, 3

Treatment Algorithm by Disease Severity

Mild-to-Moderate Non-Necrotizing Anterior Scleritis

• Start with oral NSAIDs (e.g., indomethacin, ibuprofen) as initial therapy 1, 2
• Only 16.7% of episcleritis cases require more than topical therapy, but scleritis nearly always demands systemic treatment 5
• Escalate to oral prednisone if NSAIDs fail after 1-2 weeks or if moderate-to-severe disease at presentation 1
• Use a tapering regimen over 6-8 weeks based on clinical response 1

Moderate-to-Severe or Necrotizing Scleritis

• Immediate systemic corticosteroids are required: oral prednisone at high doses or IV methylprednisolone for severe cases 1, 2
• Concurrent initiation of steroid-sparing immunomodulatory therapy (DMARDs) is essential 1, 3
• Necrotizing scleritis carries 91.7% risk of ocular complications and requires aggressive systemic immunosuppression 5
• 26.1% of scleritis patients overall require systemic immunosuppressive drugs, rising to 90% for necrotizing disease 5

Refractory or Severe Disease

• Anti-TNF biologics (infliximab or adalimumab) are recommended when conventional DMARDs fail or for severe necrotizing scleritis 1, 2
• Rituximab shows increasing evidence for refractory cases 4, 3
• Other immunomodulatory options include antimetabolites (methotrexate, mycophenolate mofetil), calcineurin inhibitors, and alkylating agents 2, 3

Posterior Scleritis Considerations

• Posterior scleritis is frequently missed due to subtle clinical signs and protean manifestations 3
• 85.7% develop ocular complications and 100% require oral corticosteroids or immunosuppressive drugs 5
• Look for choroidal folds, serous retinal detachment, optic disc edema, and fluid in sub-Tenon space (T-sign on ultrasonography) 6

Monitoring and Coordination

• Close coordination between ophthalmology and rheumatology/internal medicine is essential 1
• Follow-up every 1-2 weeks during acute phase, then monthly during maintenance therapy 1
• Monitor for vision-threatening complications: 58.8% of scleritis patients develop ocular complications and 15.9% experience decreased visual acuity 5

Critical Pitfalls to Avoid

• Do not confuse scleritis with episcleritis: severe pain immediately raises concern for scleritis requiring systemic therapy 1
• Do not delay ophthalmology referral: scleritis can rapidly progress to vision loss 1
• Do not rely on topical therapy alone: only 30.4% of scleritis patients respond adequately to NSAIDs, and most require systemic corticosteroids (31.9%) or immunosuppressives (26.1%) 5
• Do not miss underlying systemic disease: up to 50% have associated autoimmune conditions, and scleritis may precede systemic diagnosis, especially in vasculitis 4, 3, 7

Prognosis and Long-term Management

• Scleritis confers increased mortality risk in rheumatoid arthritis patients 7
• Necrotizing scleritis and posterior scleritis have the highest complication rates and require the most aggressive therapy 5
• Corticosteroid-sparing immunosuppressive therapies are crucial for long-term control and prevention of recurrence 3