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Scleritis: A Complete Guide to Understanding This Painful Eye Inflammation

What is Scleritis?

Scleritis is a serious, often painful, inflammatory disease of the sclera – the white, outer coating of the eye that gives the eye its shape and protects its inner structures. Unlike the more common and usually mild episcleritis, which involves only the thin superficial layer of the sclera, scleritis affects deeper vascular tissue and can threaten vision if left untreated.

The condition can be diffuse (affecting the entire sclera), nodular (localized, raised lesions), or posterior (behind the eye, harder to see). It typically presents with intense, deep aching pain that may worsen with eye movement, exposure to bright light, or wind.

Because scleritis is most often linked to systemic autoimmune disorders, it serves as an important ocular “window” into broader health issues.

Common Causes

In most cases, scleritis is not caused by an infection but by an abnormal immune response. Below are the most frequently reported associations (both autoimmune and other conditions):

• Rheumatoid arthritis (RA) – the leading systemic disease linked to scleritis.
• Granulomatosis with polyangiitis (GPA, formerly Wegener’s) – a small‑vessel vasculitis.
• Systemic lupus erythematosus (SLE) – a multi‑system autoimmune disease.
• Polyarteritis nodosa – inflammation of medium‑sized arteries.
• Inflammatory bowel disease (Crohn’s disease, ulcerative colitis).
• Sjogren’s syndrome – primarily affects moisture‑producing glands but can involve the eye.
• Relapsing polychondritis – an inflammatory condition of cartilage and connective tissue.
• Infectious agents (less common) – herpes simplex virus, herpes zoster, mycobacteria, syphilis, or fungal infections can mimic or trigger scleritis.
• Drug‑induced – bisphosphonates (e.g., alendronate) and certain immune checkpoint inhibitors have been reported to cause scleral inflammation.
• Idiopathic – up to 30 % of cases have no identifiable systemic disease after thorough evaluation.

Associated Symptoms

While the hallmark of scleritis is deep ocular pain, patients often experience a constellation of additional signs that help differentiate it from other eye conditions:

• Redness that appears deep‑violet or bluish rather than the bright pink of conjunctivitis.
• Severe, boring ache that may radiate to the forehead, jaw, or temples.
• Photophobia (sensitivity to light) and tearing.
• Blurred or decreased vision if inflammation involves the adjacent uvea (scleritis‑uveitis complex).
• Swelling of the eyelids or “chemosis” (conjunctival edema).
• Feeling of a foreign body or gritty sensation.
• In nodular scleritis, hard, raised nodules that may be tender to touch.
• Systemic symptoms such as joint pain, fever, fatigue, or skin lesions when an underlying autoimmune disease is present.

When to See a Doctor

Scleritis rarely resolves on its own and can progress rapidly. Prompt ophthalmologic evaluation is needed when any of the following occur:

• Intense, persistent eye pain lasting more than 24 hours.
• Redness that does not improve with artificial tears or over‑the‑counter anti‑inflammatories.
• Sudden change in vision (blurriness, dark spots, or loss of visual acuity).
• Pain that worsens with eye movement or exposure to wind.
• History of autoimmune disease coupled with new eye symptoms.
• Any sign of swelling, nodules, or pus-like discharge from the eye.

Delaying care can lead to complications such as scleral thinning, perforation, secondary glaucoma, or permanent vision loss.

Diagnosis

Diagnosing scleritis is a multi‑step process that combines a detailed history, a thorough eye exam, and targeted systemic work‑up.

Ophthalmic Examination

• Slit‑lamp biomicroscopy – the gold‑standard tool; reveals deep scleral injection, nodules, and the extent of inflammation.
• Visual acuity testing – to detect any loss of vision.
• Intra‑ocular pressure measurement – to rule out secondary glaucoma.
• Fundus examination – to look for posterior involvement or associated uveitis.
• Ultrasound B‑scan – useful for posterior scleritis that cannot be seen directly.

Laboratory and Imaging Studies

• Complete blood count (CBC) and erythrocyte sedimentation rate (ESR) or C‑reactive protein (CRP) – markers of systemic inflammation.
• Auto‑antibody panels: rheumatoid factor (RF), anti‑CCP, ANCA (c‑ANCA, p‑ANCA), ANA, anti‑dsDNA – help pinpoint an underlying autoimmune disease.
• Serologies for infections when indicated (syphilis, tuberculosis, herpes viruses).
• Chest X‑ray or CT scan if granulomatosis with polyangiitis is suspected.

Differential Diagnosis

Conditions that can mimic scleritis include episcleritis, acute anterior uveitis, conjunctivitis, orbital cellulitis, and corneal ulcer. Proper slit‑lamp assessment is essential to avoid misdiagnosis.

Treatment Options

Therapy aims to control inflammation, treat any underlying systemic disease, and preserve vision. Management is usually tiered, beginning with the least aggressive medication and escalating as needed.

Medical Treatments

• Non‑steroidal anti‑inflammatory drugs (NSAIDs) – oral ibuprofen or naproxen can be tried for mild, non‑nodular cases, but many patients require stronger agents.
• Corticosteroids
  • Topical drops* (e.g., prednisolone acetate 1 %) – useful for superficial involvement, often combined with a cycloplegic.
  • Periocular (sub‑Tenon) or orbital steroid injection – delivers high concentrations to the sclera while limiting systemic exposure.
  • Systemic oral steroids (prednisone 0.5–1 mg/kg/day) – indicated for moderate‑to‑severe disease or when nodules are present.
• Immunosuppressive or biologic agents – required for steroid‑resistant disease or when an underlying autoimmune disorder is active.
  • Antimetabolites: methotrexate, azathioprine, mycophenolate mofetil.
  • Calcineurin inhibitors: cyclosporine, tacrolimus.
  • Biologics: infliximab, adalimumab, rituximab – especially effective for granulomatosis with polyangiitis or refractory rheumatoid arthritis.
• Antibiotics or antiviral agents – only when an infectious etiology is confirmed (e.g., oral valacyclovir for herpes zoster scleritis).

Adjunctive & Home Care Measures

• Artificial tears or lubricating ointments to relieve secondary dryness.
• Cold compresses (10‑15 minutes, several times a day) may ease discomfort.
• Protective sunglasses to reduce photophobia and wind exposure.
• Avoid rubbing the eyes; this can worsen inflammation.
• Adhere to a regular follow‑up schedule (often every 1–2 weeks initially).

When Surgical Intervention May Be Needed

Rarely, severe scleral thinning can lead to perforation. In such cases, a scleral patch graft or tissue‑glue repair performed by an ocular surgeon may be necessary.

Prevention Tips

Because many cases are linked to systemic disease, total prevention isn’t possible, but risk can be lowered by managing underlying conditions and protecting the eyes.

• Maintain regular follow‑up with your rheumatologist or internist if you have an autoimmune disease.
• Adhere strictly to prescribed systemic medications; never stop steroids or immunosuppressants abruptly.
• Manage systemic inflammation through a balanced diet, regular exercise, and smoking cessation.
• Use protective eyewear when exposed to wind, dust, or bright sunlight.
• Promptly treat any ocular infection; early antimicrobial therapy can prevent secondary inflammation.
• Report new eye symptoms to your eye doctor immediately, especially if you already have a known connective‑tissue disease.

Emergency Warning Signs

If you experience any of the following, seek emergency ophthalmic care (e.g., emergency department or urgent‑care eye clinic) without delay:

• Sudden, severe eye pain that does not improve with prescribed medication.
• Rapid loss of vision or a “shadow/curtain” effect over part of the visual field.
• Marked swelling of the eye that makes it difficult to open.
• Visible white or yellow discharge suggesting a secondary infection.
• Signs of scleral thinning or a “bulge” in the white of the eye (suggesting impending perforation).
• Fever, chills, or systemic upset combined with eye pain, indicating possible infectious scleritis.

Key Take‑aways

Scleritis is a potentially sight‑threatening inflammation of the eye’s white outer coating, most often linked to systemic autoimmune diseases. Early recognition, comprehensive evaluation, and aggressive treatment—usually with steroids and, when needed, immunosuppressive or biologic agents—are essential to prevent complications. If you notice deep eye pain, persistent redness, or any change in vision, contact an eye‑care professional promptly.

For further reading, reputable sources include:

• Mayo Clinic – Scleritis
• American Academy of Ophthalmology – Scleritis Overview
• Cleveland Clinic – Scleritis
• National Eye Institute (NEI) – Scleritis Information

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