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Ophthalmology & Internal Medicine & Family Medicine

Sclérite | Clinique Omicron Québec

Scleritis is a severe inflammation of the sclera - the tough, white fibrous tunic that forms the outer envelope of the eye - characterized by deep + intense + often nocturnal + ocular pain radiating to the forehead + temples or jaws + and a characteristic purplish-red eye with dilation of the deep episcleral vessels (scleral vascular plexus) not whitened by topical vasoconstrictors (unlike episcleritis). Scleritis is a relative ophthalmological emergency: without prompt and appropriate treatment, it can lead to thinning and perforation of the sclera (scleromalacia + necrotizing scleritis) → risk of eyeball loss. Its fundamental feature is its frequent association - in 40 to 50 % of cases - with an underlying systemic disease, most often an autoimmuneimmune disease (rheumatoid arthritis [most common - 25-30 %] + small-vessel vasculitides [granulomatosis with polyangiitis - GPA + microscopic polyangiitis] + systemic lupus erythematosus + ankylosing spondylitis + Behçet's disease + relapsing polychondritis + Sjögren's syndrome) + or infection (ophthalmic herpes zoster + tuberculosis + syphilis + bacterial infections postbacterial infections). The discovery of scleritis therefore systematically calls for an etiological work-up in search of an underlying systemic disease - as scleritis may be the inaugural manifestation of a previously undiagnosed vasculitis or autoimmune disease - and its treatment must target both the local ocular inflammation and the causative systemic disease.

Classification and clinical forms

  • Diffuse anterior scleritis : the most frequent form (40 % of scleritis) + diffuse erythema + purplish over the entire anterior face + moderate to severe pain + sclerotic edema + good response to oral NSAIDs
  • Nodular anterior scleritis : single or multiple sclerotic nodule(s) + dark red + firm + immobile (distinguished from nodular episcleritis, in which the nodule is mobile) + marked pain + response to NSAIDs but often requires corticosteroids
  • Necrotizing anterior scleritis with inflammation : the most severe and destructive form + areas of scleral ischemia + necrosis + thinning + very intense pain + almost constant association with severe systemic disease (GPA + severe erosive RA) + systemic corticosteroids + mandatory immunosuppressants (cyclophosphamide + methotrexate + rituximab)
  • Perforating scleromalacia (necrotizing scleritis without inflammation): progressive, painless sclerotic thinning (paradox) + uvea visible under thinned sclera (blue-grey tint) + associated with long-standing, severe RA + risk of perforation at the slightest trauma + no proven effective treatment + surgery if perforation imminent
  • Posterior scleritis : rare + difficult to diagnose (not visible to the naked eye) + deep eye pain + periorbital headache + visual impairment + proptosis + restricted eye movements + exophthalmos + B-echo (T-sign - perisclerotic fluid + posterior sclerotic thickening) + orbital MRI + associated with autoimmune diseases + treatment: systemic corticosteroids ++ + immunosuppressants

Scleritis vs. episcleritis - key clinical distinction

Criteria Sclerite Episclerite
Affected layer Sclera (deep layer) Episclera (surface layer between conjunctiva and sclera)
Pain Deep + intense + nocturnal + radiating to forehead/jaw Mild + discomfort + no deep pain + or no pain at all
Eye color Purplish red + bluish (deep dilated vessels) Bright red (superficial episcleral vessels)
Phenylephrine test 10 % NO whitening → deep vessels (unreachable) Whitening in 10-15 min → superficial vessels (vasoconstricted)
Nodule (if present) Immobile + adherent to the sclera Mobile + moves with conjunctive
Systemic disease Associated in 40-50 % of cases → systematic assessment Associated in 30 % of cases → assessment if recurrent
Risk of complications High (perforation + loss of globe) Low + frequent spontaneous resolution
Treatment Oral NSAIDs + systemic corticosteroids + immunosuppressants depending on form Artificial tears + topical NSAIDs ± short-acting oral NSAIDs

Systematic etiological workup

  • Basic balance : CBC + ESR + CRP + creatinine + ionogram + liver function tests + proteinuria + urine sediment (hematic cylinders → vasculitis) + chest X-ray
  • Autoimmune test : ANA + anti-native DNA + ANCA (c-ANCA/anti-PR3 → GPA + p-ANCA/anti-MPO → PAM) + RF (rheumatoid factor) + anti-CCP (RA) + complement C3 + C4 + CH50 (lupus) + cryoglobulins + HLAB27 (SpA)
  • Infectious diseases : QuantiFERON-TB Gold (tuberculosis) + syphilis serology (TPHA + VDRL) + HIV serology + VZV serology if suggestive context
  • Imaging : thoracic HRCT scan if GPA or suspected sarcoidosis + orbital MRI if posterior scleritis

Treatment

  • Oral NSAIDs (mild to moderate forms - diffuse or nodular scleritis) : ibuprofen 400-600 mg × 3/day + or flurbiprofen (Froben®) 100 mg × 3/day + or indomethacin 25-50 mg × 3/day × 4-6 weeks + with gastric protection (PPI) + effective in 60-70 % of non-necrotizing forms
  • Systemic corticosteroids (severe + necrotizing + posterior forms) : prednisone 1 mg/kg/day × 4-6 weeks + gradual decrease + methylprednisolone IV 1 g/day × 3 days if very severe form (bolus) before oral relay + never use topical corticoids alone (ineffective on scleritis - layer too deep)
  • Immunosuppressants (necrotizing scleritis + severe systemic disease) : methotrexate 15-25 mg/week (RA + sarcoidosis) + cyclophosphamide IV (GPA + PAM + severe vasculitis) + rituximab (refractory GPA + severe RA) + mycophenolate mofetil (lupus) + treatment of causative systemic disease as a priority
  • Infectious scleritis: antivirals (aciclovir + valaciclovir for VZV) + antituberculosis drugs if tuberculosis + penicillin G if syphilis → never treat scleritis empirically with corticosteroids alone without first ruling out infection (risk of worsening infection)
  • Surgical treatment (perforating scleromalacia + perforation) : scleral patch (connective tissue graft + fascia + donor sclera) + vitrectomy if endocular complications + complex surgery + variable results
ℙ️ Granulomatosis with polyangiitis (GPA - formerly Wegener's disease) is the most serious systemic cause associated with necrotizing scleritis - and scleritis may be the first manifestation of this life-threatening vasculitis (renal + pulmonary involvement). Any patient with necrotizing scleritis should have an urgent ANCA assay (c-ANCA/anti-PR3) + urine sediment analysis + creatinine + chest X-ray to exclude early GPA renal and pulmonary involvement.
Urgent ophthalmological consultation

Consult an ophthalmologist urgently within 24-48 hours if a purplish-red eye appears + painful + with deep pain radiating to the forehead or jaw - these signs distinguish scleritis from simple conjunctivitis or episcleritis, and require ophthalmological management and systemic assessment without delay. Necrotizing or posterior scleritis is a strict ophthalmological emergency. For rapid referral to ophthalmology and initiation of an etiological workup (ANCA + FR + ANA + QuantiFERON), Clinique Omicron offers consultations at its points of service in Quebec and via telemedicine. To book an appointment, visit cliniqueomicron.ca.

Consult at Clinique Omicron

Clinique Omicron's specialized physicians and nurse practitioners (NPs) distinguish scleritis from episcleritis and conjunctivitis on clinical examination, immediately refer any confirmed scleritis to ophthalmology, initiate a systematic etiological workup (autoimmune + infectious + ANCA), prescribe oral NSAIDs for mild forms while awaiting specialized evaluation, and coordinate with rheumatology and internal medicine for treatment of underlying systemic disease. Consultations are available at several points of service in Quebec, and via telemedicine. To book an appointment, visit cliniqueomicron.ca.

The contents of this page are provided for information purposes only and do not replace the advice of an ophthalmologist. Scleritis is a relative ophthalmological emergency requiring rapid specialist management to prevent serious complications (perforation + loss of globe). Never use topical corticoids alone in scleritis - they are insufficient to treat deep scleral inflammation.

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