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Rheumatoid scleritis — Clinical Guidelines

Overview

Scleritis is a severe, painful inflammation of the sclera, the white outer coat of the eye, which can lead to significant ocular morbidity including vision loss, uveitis, glaucoma, and in rare cases, globe perforation. It often signifies an underlying systemic disease, particularly in older adults where rheumatoid arthritis (RA) and granulomatosis with polyangiitis (GPA) are more frequently implicated compared to younger populations. Given its potential for serious complications, early recognition and appropriate management are crucial in day-to-day practice to prevent irreversible visual impairment 1 2.

Pathophysiology

The pathophysiology of scleritis involves complex interactions between immune mediators and the sclera's unique connective tissue composition. Inflammatory cytokines, such as TNF-α and IL-1β, activate macrophages and fibroblasts within the sclera, leading to increased vascular permeability and edema. This process can disrupt the structural integrity of the sclera, characterized by vascular engorgement and nodular elevations when visible. In autoimmune contexts, such as RA, autoantibodies target ocular tissues, exacerbating inflammation. Additionally, the avascular nature of the sclera means it relies on diffusion from adjacent tissues for nutrients, making it particularly vulnerable to ischemic damage during severe inflammation 2.

Epidemiology

Scleritis has an estimated prevalence of around 6 cases per 10,000 people, though incidence rates can vary based on referral patterns and clinic settings. It predominantly affects adults, with a mean age of onset often above 40 years, particularly in older adults where systemic comorbidities like RA and GPA are more prevalent. Females are slightly more commonly affected than males. Geographic and ethnic variations are less emphasized in the literature, but certain systemic diseases associated with scleritis may show regional clustering. Over time, trends suggest an increasing recognition due to better diagnostic tools and heightened awareness of systemic associations 1 7.

Clinical Presentation

Patients with scleritis typically present with severe ocular pain, often out of proportion to the clinical findings, and may describe tenderness upon palpation of the sclera. The affected eye may exhibit redness, swelling, and a violaceous hue due to engorged episcleral vessels. Nodular scleritis presents with palpable nodules, while diffuse scleritis shows more generalized involvement. Vision changes can range from minimal to severe, depending on the extent of inflammation and associated complications such as uveitis or glaucoma. Red-flag features include sudden vision loss, systemic symptoms like fever, and signs of systemic disease, which necessitate urgent evaluation 1 2.

Diagnosis

Diagnosis of scleritis involves a comprehensive clinical assessment and targeted investigations to rule out mimics and identify underlying causes. Key diagnostic criteria include:

Clinical Examination: Presence of scleral tenderness, violaceous discoloration, and characteristic nodules or diffuse thickening.

Laboratory Tests:

- Complete Blood Count (CBC): Elevated white blood cell count may indicate infection or inflammation. - Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP): Elevated levels suggest active inflammation. - Autoantibody Panel: Useful in diagnosing associated autoimmune conditions like RA or SLE.

Imaging:

- B-Scan Ultrasonography: Can reveal fluid accumulation in the sub-Tenon’s space in posterior scleritis.

Differential Diagnosis:

- Episcleritis: Typically less painful, with blanching on phenylephrine instillation. - Scleromalacia Perforans: Usually painless, with characteristic thinning and blue-gray discoloration. - Infectious Causes: History of trauma or surgery, poor response to steroids, and positive cultures can differentiate 1 2 7.

Management

First-Line Therapy

Non-Steroidal Anti-Inflammatory Drugs (NSAIDs):

- Dose: Indomethacin 75 mg PO TID or equivalent. - Duration: Typically 2-4 weeks. - Monitoring: Assess for efficacy and gastrointestinal side effects.

Topical Therapy:

- Prednisolone Acetate 1%: Adjunctive use for mild cases with anterior segment involvement. - Monitoring: Evaluate for local side effects like glaucoma or cataract formation.

Second-Line Therapy

Systemic Corticosteroids:

- Oral Prednisone: - Dose: Initial dose of 40-60 mg/day, tapering over weeks based on response. - Duration: Variable, often several weeks to months. - Monitoring: Regular blood glucose, blood pressure, and bone density checks. - Intravenous Methylprednisolone: - Dose: Pulse therapy of 1-2 g IV daily for 3-5 days. - Indication: Severe cases unresponsive to oral steroids. - Monitoring: Hypertensive response, infection risk, adrenal suppression.

Refractory or Specialist Escalation

Disease-Modifying Anti-Rheumatic Drugs (DMARDs):

- Methotrexate: - Dose: 7.5-20 mg/week PO. - Indication: For underlying RA or other autoimmune conditions. - Monitoring: Liver function tests, complete blood count, renal function. - Biologics (e.g., Anti-TNF agents): - Dose: Infliximab 5 mg/kg IV every 6-8 weeks. - Indication: Severe refractory cases with underlying autoimmune disease. - Monitoring: Infusion reactions, infection risk, and efficacy markers.

Infectious Causes:

- Antibiotics: Broad-spectrum initially, tailored based on culture and sensitivity. - Surgical Debridement: May be necessary in cases of refractory infection.

Contraindications

Active Infection: Avoid systemic steroids until infection is controlled.

Severe Hypertension/Renal Failure: Careful monitoring and dose adjustment required for corticosteroids.

Complications

Vision Loss: Due to uveitis, glaucoma, or globe perforation.

Glaucoma: Secondary angle-closure or open-angle glaucoma from inflammation.

Necrotizing Scleritis: Rare but severe, leading to scleromalacia perforans.

Systemic Complications: Worsening of underlying systemic disease.

When to Refer: Persistent vision loss, suspicion of necrotizing scleritis, or refractory cases requiring biologic DMARDs 1 2 7.

Prognosis & Follow-Up

The prognosis of scleritis varies widely depending on the underlying cause and promptness of treatment. Patients with infectious causes generally have a poorer prognosis compared to those with autoimmune etiologies. Prognostic indicators include the presence of necrotizing scleritis, severity of initial inflammation, and response to initial therapy. Recommended follow-up intervals typically include:

Initial Follow-Up: Within 1-2 weeks post-diagnosis to assess response to therapy.

Subsequent Monitoring: Every 1-3 months initially, tapering based on clinical stability.

Long-Term Monitoring: Regular ophthalmologic evaluations and systemic disease management reviews 1 7.

Special Populations

Elderly Patients

Considerations: Increased prevalence of comorbidities like RA, hypertension, and cardiovascular disease.

Management: Tailored drug selection considering side-effect profiles and interactions with existing medications.

Monitoring: Frequent checks for systemic effects of corticosteroids and DMARDs 1.

Autoimmune Associations

Rheumatoid Arthritis (RA): Close collaboration with rheumatologists for comprehensive management.

Granulomatosis with Polyangiitis (GPA): Multidisciplinary approach including nephrology and pulmonology if systemic involvement 1 8.

Key Recommendations

Initiate NSAIDs as first-line therapy for mild to moderate scleritis, monitoring for efficacy and side effects (Evidence: Strong 2).

Use systemic corticosteroids for severe cases, with careful monitoring of systemic effects (Evidence: Strong 1).

Consider DMARDs or biologics in refractory cases with underlying autoimmune disease (Evidence: Moderate 1 8).

Perform comprehensive systemic evaluation to identify and manage underlying causes (Evidence: Strong 7).

Regular follow-up is essential to monitor both ocular and systemic health (Evidence: Moderate 1 7).

Avoid systemic steroids in active infections until infection is controlled (Evidence: Expert opinion 2).

Collaborate with rheumatology and infectious disease specialists for complex cases (Evidence: Expert opinion 1).

Monitor for complications such as vision loss and glaucoma, especially in refractory cases (Evidence: Moderate 2).

Tailor treatment in elderly patients considering comorbidities and drug interactions (Evidence: Moderate 1).

Evaluate and manage associated systemic diseases concurrently to improve overall prognosis (Evidence: Strong 7).

References

1 Butler L, Tomkins-Netzer O, Reiser O, Niederer RL. Management of Scleritis in Older Adults. Drugs & aging 2024. link 2 Beardsley RM, Suhler EB, Rosenbaum JT, Lin P. Pharmacotherapy of scleritis: current paradigms and future directions. Expert opinion on pharmacotherapy 2013. link 3 Cheruvu NP, Amrite AC, Kompella UB. Effect of eye pigmentation on transscleral drug delivery. Investigative ophthalmology & visual science 2008. link 4 Tak D, Rimpy, Kumar T, Ahuja M. Optimization of the Interaction between Diclofenac and Ibuprofen with Benzalkonium Chloride to Prepare Ocular Nanosuspension. Recent patents on drug delivery & formulation 2019. link 5 Katara R, Sachdeva S, Majumdar DK. Aceclofenac oil drops: characterization and evaluation against ocular inflammation. Pharmaceutical development and technology 2018. link 6 Zhang J, Sun H, Zhou N, Zhang B, Ma J. Preparation and Evaluation of Biodegradable Scleral Plug Containing Curcumin in Rabbit Eye. Current eye research 2017. link 7 Erkanli L, Akova YA, Guney-Tefekli E, Tugal-Tutkun I. Clinical features, prognosis, and treatment results of patients with scleritis from 2 tertiary eye care centers in Turkey. Cornea 2010. link 8 Taban M, Langston RH, Lowder CY. Scleritis in a person with stiff-person syndrome. Ocular immunology and inflammation 2007. link 9 Lu Y, Fukuda K, Seki K, Nakamura Y, Kumagai N, Nishida T. Inhibition by triptolide of IL-1-induced collagen degradation by corneal fibroblasts. Investigative ophthalmology & visual science 2003. link 10 Balasubramaniam J, Kumar MT, Pandit JK, Kant S. In vitro and in vivo characterization of scleral implants of indomethacin. Die Pharmazie 2001. link

Rheumatoid scleritis