🆘 Scleritis is a vision-threatening emergency → same-day referral to Ophthalmology.
🔴 It involves painful, destructive inflammation of the sclera, often associated with systemic disease.

🧩 Rheumatological Associations

🤲 Rheumatoid arthritis (most common)
🦠 Inflammatory bowel disease (Crohn’s, Ulcerative Colitis)
🫁 Granulomatosis with polyangiitis (Wegener’s)
🩸 Polyarteritis nodosa (PAN), Giant Cell Arteritis (GCA), Systemic Lupus Erythematosus (SLE)
🦴 Seronegative spondyloarthropathies
🎧 Relapsing polychondritis

🦠 Infective Associations

🦠 Herpes Zoster Ophthalmicus
🧫 Pseudomonas & other bacterial infections
🍄 Fungal and protozoal infections
🧬 Syphilis, Tuberculosis, Sarcoidosis

👁️ Clinical Features

⚡ Severe pain, worsens on eye movement, globe is tender.
🌙 Pain may wake patient at night; photophobia common.
👁️ Anterior scleritis: diffuse or nodular scleral erythema.
👁️ Posterior scleritis: reduced vision, retinal/choroidal/extraocular muscle oedema.
⚠️ White patches of necrosis (pallor) within erythema → suggests systemic vasculitis.
🔵 Chronic disease may cause scleral thinning (scleromalacia), exposing the blue choroid.

🔬 Investigations

🩺 Clinical diagnosis → urgent ophthalmology assessment.
🧪 Bloods: FBC, ESR/CRP, ANA, ANCA, RF, complement, syphilis serology.
🦠 Infective screen: TB, syphilis, viral PCR if relevant.
🖼️ Imaging: B-scan ultrasound/MRI orbit if posterior scleritis suspected.
🔍 Consider systemic work-up if vasculitis suspected.

🧫 Pathology

Autoimmune granulomatous inflammation with vasculitis of scleral vessels.
May progress to necrosis and scleral thinning.
Immune-complex mediated in many rheumatological causes.

👁️ Episcleritis vs Scleritis vs Scleromalacia Perforans

Feature	Episcleritis	Scleritis	Scleromalacia Perforans
🔥 Pain	Mild discomfort or none	Severe, deep, boring pain that may radiate to the jaw, brow, or temple	Painless ⚠️
🔴 Redness	Sectoral or diffuse redness	Diffuse, deep violaceous or dusky redness	Minimal redness despite severe disease
🎯 Vessel appearance	Superficial episcleral vessels	Deep scleral vessels with a purple-blue hue	Marked scleral thinning with visible underlying uveal tissue giving a blue-grey appearance
💧 Blanching with phenylephrine	Yes – superficial vessels blanch	No – deep scleral vessels do not blanch	No
👁️ Visual acuity	Usually normal	May be reduced	May be reduced
🧬 Underlying cause	Often idiopathic; may be associated with mild systemic disease	Often autoimmune, especially rheumatoid arthritis or systemic vasculitis	Strongly associated with long-standing rheumatoid arthritis
⚠️ Degree of inflammation	Mild, superficial inflammation	Marked deep inflammation	Minimal visible inflammation despite necrotising disease
🪨 Structural damage	None	Possible scleral thinning and necrosis	Severe scleral thinning with scleral “melting”
💥 Complications	Rare	Vision loss, keratitis, uveitis, scleral thinning, perforation	Globe rupture and permanent visual loss 🚨
💊 Treatment	Usually self-limiting; lubricants or oral NSAIDs if symptomatic	Systemic NSAIDs, corticosteroids, or immunosuppressive therapy depending on severity	Urgent systemic immunosuppression; may require scleral grafting
🚨 Urgency	Low	Urgent ophthalmology review	Ophthalmic emergency

🧠 Exam Tips

📌 Episcleritis is benign, superficial, and usually only mildly uncomfortable.
📌 Scleritis causes severe pain and is often linked to systemic autoimmune disease.
📌 Scleromalacia perforans is the classic painless blue sclera seen in long-standing rheumatoid arthritis.
📌 The absence of pain in scleromalacia perforans is a classic exam trap — it is often the most severe of the three.
📌 Phenylephrine blanching helps distinguish episcleritis from deeper scleral disease.

💊 Management

🔥 Necrotising scleritis → requires aggressive systemic immunosuppression (high-dose steroids + DMARDs or biologics).
🌿 Non-necrotising scleritis → topical/oral NSAIDs, topical steroids, or oral corticosteroids.
💉 Severe/refractory disease → IV steroids, immunosuppressants (methotrexate, cyclophosphamide, azathioprine), TNF-α inhibitors.
🔍 Always investigate & treat underlying systemic associations.
👩‍⚕️ Close liaison with rheumatology & ophthalmology teams.

📚 References

British Society for Rheumatology: Guidelines for the management of ocular inflammatory disease.
BMJ Best Practice: Scleritis overview.
Watson & Hayreh: Scleritis and episcleritis, Oxford University Press.

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Scleritis

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