Scleritis

 

 

 

 

 

 

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Aetiology

Scleritis is a rare but potentially severe sight-threatening inflammatory disease of the sclera which is bilateral in 50% of cases

Predisposing factors

Incidence: 3.4 per 100,000 per annum
Patients are usually in the middle age group (40-60 years)
M:F = 2:3
May be idiopathic, but approximately a half of cases are associated with autoimmune disease, of which the scleritis may be the first presentation:

• rheumatoid arthritis
• vasculitides, including granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis), polyarteritis nodosa, giant cell arteritis
• seronegative spondyloarthropathies, including ankylosing spondylitis, psoriatic arthritis
• systemic lupus erythematosus
• inflammatory bowel disease
• relapsing polychondritis

4-10% of scleritis is infectious in origin, e.g.:

• Herpes Zoster Ophthalmicus
• Pseudomonas and other bacterial infections
• fungal and protozoal infections
• syphilis
• sarcoidosis, TB

Trauma and surgery:
Surgery-induced necrotizing scleritis, SINS, is a rare complication of ocular surgery, including pterygiectomy, cataract, and scleral buckling, often associated with infection, especially by Pseudomonas

Symptoms

Moderate or severe pain (eye ‘ache’ may be referred to brow or jaw) which is exacerbated by eye movement
May disturb sleep
Gradual onset
Tenderness of globe
Photophobia
Epiphora
Visual loss
Possible history of previous episodes

Signs

Scleritis may involve the anterior sclera, the posterior sclera, or both

Anterior scleritis (90% of cases)

(a) Non-necrotising (75% of cases)

• usually unilateral
• hyperaemia of superficial and deep episcleral vessels; does not blanch with vasoconstrictors (e.g. gutt. phenylephrine 2.5%)
• anterior uveitis may be present
• tenderness of globe
• when inflammation resolved, choroidal pigment may show through thinned sclera as a blue/black colouration
• approximately 60% are diffuse and 40% nodular (scleral nodule cannot be moved over underlying tissue)

(b) Necrotising (15% of cases)

• the most severe form (may occur in the absence of pain). 75% will eventually have visual impairment
• avascular patches leading to scleral melting with ectasia and choroidal herniation

Posterior scleritis (10% of cases)

Involves sclera posterior to the ora serrata. Eye may be white.
Ophthalmoscopy may show exudative retinal detachment, macular oedema, optic disc oedema, but may also show no abnormality

Differential diagnosis

Episcleritis (see Clinical Management Guideline on Episcleritis)
Other causes of acute red eye

Management by optometrist

Practitioners should recognise their limitations and where necessary seek further advice or refer the patient elsewhere

Non pharmacological

None

Pharmacological

• Systemic analgesia (e.g. paracetamol, aspirin, ibuprofen)

(GRADE*: Level of evidence=low, Strength of recommendation=strong)

Management category

A2: first aid measures and emergency (same day) referral. Emphasise to the patient the urgency of the condition and instruct them to attend the local hospital eye department or hospital A & E the same day, explaining that you will leave a message so that they are expected. Telephone the department to explain what you have done, preferably leaving your message with a doctor or other health care professional.

No intervention except analgesia.

Possible management by ophthalmologist

Investigation for systemic associations
Topical steroid
Systemic non-steroidal anti-inflammatory drugs
Systemic immunosuppression (corticosteroids +/- other immunosuppressant drugs, and biologics, e.g. TNFalpha inhibitors)
Imaging to investigate posterior segment involvement
May require referral to, or co-management with, Physician or Rheumatologist

Evidence base

*GRADE: Grading of Recommendations Assessment, Development and Evaluation  (www.gradeworkinggroup.org)

Sources of evidence

Agrawal R, Lee CS, Gonzalez-Lopez JJ, Khan S, Rodrigues V, Pavesio C. Flurbiprofen: A Nonselective Cyclooxygenase (COX) Inhibitor for Treatment of Noninfectious, Non-necrotizing Anterior Scleritis. Ocul Immunol Inflamm. 2016;24(1):35-42

Daniel Diaz J, Sobol EK, Gritz DC. Treatment and management of scleral disorders. Surv Ophthalmol. 2016;61(6):702-717

Oray M, Meese H, Foster CS. Diagnosis and management of noninfectious immune-mediated scleritis: current status and future prospects. Expert Rev Clin Immunol. 2016;12(8):827-37

Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, Doctor PP, Tauber J, Foster CS. Clinical characteristics of a large cohort of patients with scleritis and episcleritis. Ophthalmology. 2012;119(1):43-50

Sainz de la Maza M, Molina N, Gonzalez-Gonzalez LA, Doctor PP, Tauber J, Foster CS.Jabs DA, Scleritis therapy. Ophthalmology. 2012;119(1):51-8

Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol 1976;60:163-91

Wieringa W, Wieringa JE, ten Dam-van Loon NH, Los Ll. Visual outcome, treatment results, and prognostic factors in patients with scleritis. Ophthalmology. 2013; 120(2):379-86

Plain language summary 

Scleritis is a rare, severe inflammation of the sclera (the white part of the eyeball). It affects older people, women more than men, around a third of whom have some other form of inflammation, such as rheumatoid arthritis or inflammation of the bowel, or long-standing infection elsewhere in the body. It may affect one or both eyes.

The condition begins gradually and patients experience an aching pain in the eye, which may spread to the brow region or to the jaw. This may be so severe as to cause loss of sleep. Patients may also find lights unbearably bright and the vision may be blurred.

There are two forms of Scleritis: Anterior Scleritis, which makes up 9 out of 10 cases and involves the sclera of the front part of the eye, where the inflammation (redness and swelling) can easily be seen. An even more severe form of Anterior Scleritis seen in 15% of these cases is known as Necrotising Scleritis, in which the scleral tissue melts away, often without pain; vision is likely to be greatly impaired. One in 10 cases of Scleritis takes the form known as Posterior Scleritis, which affects the sclera of the back part of the eye, so that the front of the eye may appear normal and the optometrist will need to use special instruments to help to make the diagnosis.

Scleritis is a serious condition and it is recommended that all cases be referred as emergencies to the ophthalmologist, who will usually treat the condition with drugs given by mouth that reduce inflammation and suppress the body’s immune system.

Scleritis
Version 9
Date of search 28.08.19
Date of revision 27.09.19
Date of publication 23.04.21
Date for review 27.08.21
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