A severe sight threatening inflammatory conditions characterised by inflammation of the sclera

Anterior scleritis (90% of cases)

  • Diffuse anterior - commonest (50%) and benign form - widespread inflammation of anterior sclera. Not sight threatening and tends to resolve
  • Nodular - erythematous tender fixed nodules in the sclera. 1 in 4 progress to necrotising scleritis. Commonly reoccurs
  • Necrotising - less frequent. extreme pain and marked scleral damage. Usually associated with underlying systemic disease
    • with corneal inflammation - sclerokeratitis
    • without inflammation (scleromalacia perforans) - notable for lack of symptoms until the bare choroid is seen under the thin ater of the conjunctiva. Bilateral and only seen in advanced RA usually in women

Posterior scleritis

Episcleritis

Is inflammation of the superficial episcleral later of the eye. It is relatively common benign and self-limiting. Not that painful - only a segment of the eye may be red Episcleritis does not progress to scleritis

ScleritisEpiscleritis
PathophysiologyAutoimmune dysregulationIdiopathic inflammation
SymptomsSubacute
Severe pain
Pain with eye movement
Blurred vision/vision loss
Photophobia
Acute onset
Mild pain
Redness, irritation
Physical examAdherent vessels
Does not blanch with phenylephrine drops
Bluish hue
Slit lamp may reveal nodules scleral thining and corneal changes
Systemic inflammation (joint pain Rashes)
Mobile vessels
Blanch with phenylephrine drops
Reddish hue
TreatmentSystemic steroid/NSAIDs topical antibioticsSelf-limited
Consider topical steroids in refractory cases

Causes/Factors

Symptoms

  • Severe eye pain
  • Gradual onset of severe redness
  • Photophobia
  • Tearing - excessive tearing due to ocular irritation and inflammation

Signs

  • Scleral thickening - visible on exam
  • Scleral injection - diffuse or localised injfection of the scleral vessels with a blueish hue
  • Nodules or plaques on the sclera - chronic inflammation
  • Episcleral vascular engorgement

Investigations

  • Slit-lamp to assess scleral inflammation oedema and associated complications
  • USS - to evaluate scleral and intraocular structures particularly in posterior scleritis
  • Blood tests for autoimmune and inflammatory markers. At least 50% of patients have an underlying autoimmune condition

Management

Diffuse anterior scleritis and nodular scleritis

  • Oral NSAIDs (ibuprofen 400m qds)

  • If not effective then oral Prednisolone (80mg od)

  • If not effective could try subconjuctival or periorbital steroid injection but controversial

  • If not effective immunosuppressive therapy - methotrexate, azathioprine mycophenolate, cyclophosphamide or ciclosporin may be helpful

  • Diagnostic biopsy can be helpful

  • Surgery can be required to address complications

Necrotising anterior scleritis

  • Systemic steroids and immunosuppresive therapies
  • May need surgical intervention if perforation is impending

Scleromalacia perforans

  • No specific effective treatment

Posterior scleritis

  • Younger patients usually respond well to oral NSAIDs but elderly patients as managed as with anterior scleritis

Complications/red Flags