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
Scleritis | Episcleritis | |
---|---|---|
Pathophysiology | Autoimmune dysregulation | Idiopathic inflammation |
Symptoms | Subacute Severe pain Pain with eye movement Blurred vision/vision loss Photophobia | Acute onset Mild pain Redness, irritation |
Physical exam | Adherent 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 |
Treatment | Systemic steroid/NSAIDs topical antibiotics | Self-limited Consider topical steroids in refractory cases |
Causes/Factors
- Autoimmune disorders - a/w RA is most common, Systemic Lupus Erythematosus, Inflammatory Arthritis, granulomatosis with polyangiitis
- Infections
- Trauma
- Idiopathic sometimes not known
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
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If not effective immunosuppressive therapy - methotrexate, azathioprine mycophenolate, cyclophosphamide or ciclosporin may be helpful
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Diagnostic biopsy can be helpful
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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