How should scleral swelling (scleritis or episcleritis) in an adult be evaluated and managed?

Scleral Swelling Evaluation and Management

Distinguish episcleritis from scleritis immediately at presentation, as scleritis requires aggressive systemic anti-inflammatory treatment to prevent vision loss, while episcleritis is self-limited and needs only symptomatic care.

Critical First Step: Differentiate the Two Entities

The most important clinical decision is distinguishing episcleritis from scleritis, as they have fundamentally different prognoses and treatment approaches 1, 2:

Episcleritis characteristics:

• Superficial inflammation affecting only the episclera
• Benign, self-limited course
• Minimal to mild discomfort
• Associated systemic disease in <30% of cases 3
• No risk of vision loss

Scleritis characteristics:

• Deep scleral inflammation with severe, boring pain
• Potentially sight-threatening
• Associated systemic disease in 40-50% of cases 2
• Risk of vision loss approximately 16% overall, up to 50% in necrotizing forms 4
• Requires mandatory systemic treatment

Evaluation Algorithm

Clinical Examination

Perform slit lamp examination to assess:

• Depth of vessel involvement (superficial episcleral vs deep scleral vessels)
• Pain severity (mild discomfort suggests episcleritis; severe pain indicates scleritis)
• Phenylephrine test: topical phenylephrine blanches episcleral vessels but not deep scleral vessels
• Classification if scleritis: diffuse, nodular, necrotizing with inflammation, or scleromalacia perforans 1

Mandatory Systemic Work-Up for Scleritis

All scleritis patients require comprehensive systemic evaluation 1, 2:

• Blood work: Complete blood count, inflammatory markers (ESR, CRP), rheumatoid factor, ANCA antibodies, ANA panel
• Imaging: Chest X-ray or CT to evaluate for systemic vasculitis or granulomatosis
• Infectious work-up: Consider herpes virus testing (8% of scleritis is infectious, mainly herpes viruses) 4
• Collaboration with rheumatology/internal medicine is essential

Key systemic associations to identify:

• Rheumatoid arthritis (most common, 12.8% of cases) 4
• ANCA-associated vasculitides (7.8% of cases) 4
• Granulomatosis with polyangiitis 1

Limited Work-Up for Episcleritis

Episcleritis rarely requires extensive investigation unless recurrent or atypical 3.

Treatment Algorithm

For Episcleritis (Symptomatic Only)

• Artificial tears for comfort
• Topical corticosteroids if needed for symptom relief
• Oral NSAIDs for persistent discomfort 3
• Self-limited course; no systemic immunosuppression needed

For Scleritis (Aggressive Stepwise Approach)

The treatment intensity must match the severity, with necrotizing forms requiring the most aggressive therapy 5, 3:

1. First-line: Oral NSAIDs for mild, non-necrotizing cases

2. Second-line: Systemic corticosteroids (oral prednisone 1 mg/kg/day) for moderate cases or NSAID failures 1, 6

3. Third-line: Disease-modifying anti-rheumatic drugs (DMARDs) or immunosuppressants for:

   • Necrotizing scleritis
   • Corticosteroid-refractory cases
   • Associated systemic autoimmune disease
   • Patients requiring long-term treatment 2, 4

4. Biologic agents: Consider for refractory or complicated cases 2

Treatment must be initiated early and aggressively to prevent irreversible vision loss and reduce mortality risk 1.

Critical Pitfalls

• Never treat scleritis with topical therapy alone—systemic inflammation requires systemic treatment
• Necrotizing scleritis has 80% association with systemic disease and 50% risk of vision loss—these patients need immediate aggressive immunosuppression 4
• Scleromalacia perforans (necrotizing without inflammation) occurs in long-standing rheumatoid arthritis and has the worst prognosis 5
• In older adults, carefully weigh medication side effects, particularly with corticosteroids and immunosuppressants, given increased risk of complications 6
• Posterior scleritis has higher complication rates and requires imaging for diagnosis 3

Special Considerations

Infectious scleritis (5-10% of cases) requires targeted antimicrobial therapy, not immunosuppression—this is why infectious work-up is mandatory before starting immunosuppressive treatment 2.

The principle is early, individualized, and aggressive treatment based on severity and underlying systemic disease 5. Scleritis management requires expertise in immunosuppressive therapy, as approximately 50% of patients will need these agents 4.