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Scleritis

Specialties > Ophthalmology

Background knowledge 🧠

Definition

  • Scleritis is a severe, painful inflammatory disease affecting the sclera, the white outer coating of the eye.
  • It can lead to significant ocular morbidity and vision loss if untreated.
  • Associated with systemic autoimmune conditions.

Epidemiology

  • Incidence: Rare, with an estimated 4 cases per 100,000 population per year.
  • Age: Typically affects adults aged 30-60 years.
  • Gender: More common in females.
  • Associated with systemic autoimmune diseases in up to 50% of cases.

Aetiology and Pathophysiology

  • Autoimmune conditions: Rheumatoid arthritis, granulomatosis with polyangiitis (Wegener’s).
  • Infectious causes: Herpes zoster, tuberculosis, syphilis.
  • Idiopathic in many cases, where no underlying cause is identified.
  • Pathophysiology involves immune complex deposition, leading to inflammation and tissue damage.

Types

  • Anterior Scleritis: More common, involves the front part of the sclera.
  • Posterior Scleritis: Involves the back part of the sclera, may present with less visible redness.
  • Diffuse, nodular, and necrotising are subtypes based on clinical appearance and severity.
  • Necrotising scleritis has the worst prognosis, often leading to scleral thinning and perforation.

Clinical Features 🌡️

Symptoms

  • Severe, deep ocular pain, often radiating to the forehead or jaw.
  • Redness of the eye, which may be localized or diffuse.
  • Photophobia and tearing.
  • Visual disturbances in severe cases, such as blurred vision.
  • Pain that worsens with eye movement.

Signs

  • Redness, typically involving the deeper episcleral vessels.
  • Tenderness of the globe on palpation.
  • Nodules may be present in nodular scleritis.
  • In necrotising scleritis, scleral thinning or perforation may be visible.
  • Posterior scleritis may present with proptosis, restricted eye movement, or choroidal folds.

Investigations 🧪

Tests

  • Slit-lamp examination: Confirms diagnosis and differentiates from episcleritis.
  • Blood tests: ESR, CRP, rheumatoid factor, and ANCA to assess for underlying systemic disease.
  • Imaging: B-scan ultrasonography for posterior scleritis, MRI if orbital involvement suspected.
  • Microbiological studies: If infection is suspected, perform cultures or PCR.
  • Biopsy: Rarely needed but can be done if malignancy is suspected.

Management 🥼

Management

  • Non-steroidal anti-inflammatory drugs (NSAIDs) for mild cases.
  • Systemic corticosteroids are first-line for more severe cases.
  • Immunosuppressants: Methotrexate, cyclophosphamide for resistant or severe cases.
  • Treat underlying systemic disease if identified.
  • Surgical intervention: Rare, used for complications such as scleral perforation.

Complications

  • Scleral thinning and perforation, particularly in necrotising scleritis.
  • Glaucoma due to raised intraocular pressure.
  • Cataract formation from prolonged steroid use.
  • Vision loss due to corneal involvement or secondary uveitis.
  • Orbital cellulitis in cases of secondary infection.

Prognosis

  • Variable depending on underlying cause and response to treatment.
  • Better prognosis in non-necrotising cases with prompt treatment.
  • Poorer prognosis in necrotising scleritis due to higher risk of complications.
  • Chronic and relapsing course common in autoimmune-related scleritis.
  • Long-term follow-up needed to monitor for recurrence or complications.

Key Points

  • Scleritis is a serious condition requiring prompt recognition and treatment.
  • Always consider an underlying systemic disease.
  • Differentiate from episcleritis, which is less severe and self-limiting.
  • Management involves systemic treatment rather than topical therapies.
  • Monitor for complications and long-term sequelae.

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Ophthalmology