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Nodular Scleritis (Scleritis)

Overview

What is it? Nodular scleritis is a painful, inflammatory condition of the sclera—the tough, white outer coating of the eye. It is a subtype of anterior scleritis in which one or more localized, raised nodules form on the scleral surface. The inflammation can be focal (single nodule) or diffuse (multiple nodules).

Who it affects? Scleritis most commonly occurs in adults aged 30–60 years and is slightly more prevalent in women (≈ 55‑60%). Nodular scleritis accounts for roughly 25‑30 % of all anterior scleritis cases.<sup>[1]</sup>

Prevalence Overall scleritis is relatively rare, affecting about 3–4 per 100,000 people annually in the United States.<sup>[2]</sup> Because nodular scleritis is only a subset, its incidence is estimated at 0.8–1.2 per 100,000 per year.

Symptoms

Symptoms can develop rapidly (over hours) or progress slowly over days. They may be unilateral (one eye) or, less commonly, bilateral. Common features include:

• Severe, deep‑seated eye pain – often described as a deep, boring ache that worsens with eye movement, exposure to wind, or bright light.
• Visible nodule(s) – a raised, pinkish‑red or bluish lump on the sclera, sometimes with overlying conjunctival injection.
• Redness (hyperemia) – intense circumferential or localized redness around the nodule.
• Tearing and photophobia – increased tearing and sensitivity to light.
• Blurred vision – usually mild, but can worsen if the inflammation extends to the cornea or retina.
• Headache or facial pain – referred pain can radiate to the forehead or jaw.
• Eye floaters – occasional perception of specks if inflammation involves the vitreous.
• Reduced eye movement – discomfort during up‑ or down‑gaze.

Causes and Risk Factors

Underlying Causes

Nodular scleritis is usually a manifestation of systemic disease rather than a primary eye problem. The most common associations are:

• Autoimmune / Connective‑tissue disorders – Rheumatoid arthritis (≈ 30‑40 % of cases), granulomatosis with polyangiitis (Wegener’s), systemic lupus erythematosus, relapsing polychondritis, and Sjögren’s syndrome.<sup>[3]</sup>
• Infectious agents – Bacterial (e.g., Staphylococcus aureus, Treponema pallidum), viral (herpes simplex, varicella‑zoster), fungal (Histoplasma, Candida) and parasitic (e.g., Onchocerca) infections can provoke a nodular reaction.
• Idiopathic – In up to 20‑30 % of cases no systemic cause is identified despite thorough work‑up.

Risk Factors

• Existing autoimmune disease (especially rheumatoid arthritis)
• History of previous scleritis or uveitis
• Use of systemic immunosuppressants that may mask early infection
• Older age (peak incidence 40‑55 years)
• Female gender (slightly higher risk)

Diagnosis

Early and accurate diagnosis is essential to prevent vision‑threatening complications.

Clinical Evaluation

1. History – Detailed symptom chronology, systemic disease review, medication list, and exposure to infections.
2. Slit‑lamp examination – The definitive tool; the ophthalmologist will observe the characteristic nodular lesion, scleral thickening, and overlying conjunctival injection.
3. Visual‑acuity testing – Baseline measurement for future comparison.
4. Fundus examination – To rule out posterior segment involvement.

Ancillary Tests

• Blood work – CBC, ESR, CRP, rheumatoid factor, anti‑CCP, ANA, ANCA, complement levels, and specific auto‑antibodies based on clinical suspicion.
• Infectious work‑up – VDRL/RPR for syphilis, TB skin test or IGRA, viral PCR (HSV, VZV), fungal serologies if indicated.
• Imaging
  • Orbital ultrasound or B‑scan – shows scleral thickening and helps differentiate from episcleritis.
  • Orbital MRI with contrast – useful when posterior extension is suspected.
• Biopsy – Rarely required, but in atypical or refractory cases a scleral biopsy can distinguish infectious from immune‑mediated inflammation.

Treatment Options

Treatment aims to control inflammation, preserve vision, and address any underlying systemic disease.

Medications

• Topical steroids – Usually insufficient for nodular scleritis but may be added for mild cases.
• Systemic non‑steroidal anti‑inflammatory drugs (NSAIDs) – First‑line for mild, non‑necrotizing disease (e.g., naproxen 500 mg BID). Effectiveness is limited in nodular forms.
• Systemic corticosteroids – Oral prednisone 1 mg/kg/day (max 60 mg) with gradual taper over 4–6 weeks is standard for moderate‑to‑severe disease.
• Immunosuppressive agents (steroid‑sparing):
  • Methotrexate 15‑25 mg weekly
  • Mycophenolate mofetil 1–2 g BID
  • Azathioprine 2–2.5 mg/kg/day
  • Cyclosporine 3–5 mg/kg/day (monitor renal function)
  These are started when long‑term steroids are needed or if disease recurs during taper.
• Biologic therapies – For refractory cases or when associated with certain systemic diseases:
  • Rituximab (anti‑CD20) – especially in rheumatoid‑associated scleritis.
  • Tumor necrosis factor (TNF) inhibitors (infliximab, adalimumab) – useful in granulomatosis with polyangiitis.
• Antibiotic/antiviral/antifungal therapy – Directed against identified infectious agents (e.g., IV ceftriaxone for syphilis, oral acyclovir for HSV).

Procedural Interventions

• Periocular (sub‑tenon) steroid injection – Triamcinolone acetonide 40 mg can provide rapid relief when systemic therapy is contraindicated.
• Debulking surgery – Rare, performed only for necrotic scleral nodules that threaten globe integrity.

Lifestyle & Supportive Measures

• Protect eyes from wind, bright sunlight, and dust (wrap‑around sunglasses).
• Use preservative‑free artificial tears 4–6 times daily to relieve surface irritation.
• Maintain a balanced diet rich in omega‑3 fatty acids (found in fish, flaxseed) – modest anti‑inflammatory benefit.
• Quit smoking; nicotine worsens autoimmune eye inflammation.
• Regular follow‑up with both an ophthalmologist and the physician managing any systemic disease.

Living with Nodular Scleritis (Scleritis)

Daily Management Tips

• Medication adherence – Set alarms or use a pill‑box to avoid missed doses, especially during steroid tapers.
• Monitor pain – Keep a daily diary of pain intensity (0–10 scale) and note triggers; share this with your doctor.
• Eye‑care hygiene – Wash hands before applying drops or ointments; avoid rubbing the affected eye.
• Protective eyewear – Polarized sunglasses with UV protection when outdoors; goggles for windy or dusty environments.
• Regular vision checks – At least every 3–4 months, or sooner if symptoms change.
• Manage systemic disease – Keep rheumatology appointments, comply with disease‑modifying drugs, and maintain routine labs (CBC, LFTs, renal function).
• Stress reduction – Chronic inflammation can be aggravated by stress; consider meditation, gentle yoga, or counseling.

Psychosocial Support

Living with a painful, potentially vision‑threatening condition can be stressful. Support groups (online or local) for autoimmune eye disease, and counseling services can improve coping and quality of life.

Prevention

Because many cases are linked to systemic autoimmune disease, primary prevention focuses on early detection and control of those conditions.

• Prompt diagnosis and treatment of rheumatoid arthritis, lupus, or vasculitis can lower the risk of ocular complications.
• Regular ophthalmic screening for patients with known autoimmune disorders (at least annually).
• Vaccinations (influenza, pneumococcal, shingles) to reduce infection‑related triggers.
• Good ocular hygiene and avoidance of trauma or exposure to contaminated water (especially for individuals with a history of ocular infection).

Complications

If left untreated or inadequately controlled, nodular scleritis may lead to serious outcomes:

• Sight‑threatening necrosis – Progressive scleral thinning, leading to perforation.
• Secondary glaucoma – Inflammatory debris can obstruct aqueous outflow.
• Corneal involvement – Adjacent keratitis, ulceration, or scarring causing permanent visual loss.
• Uveitis – Inflammation can spread to the uveal tract.
• Systemic disease flare – Uncontrolled ocular inflammation may reflect or precipitate systemic disease activity.

When to Seek Emergency Care

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References

1. Watson PG, et al. “Scleritis: A review of clinical features and management.” Ophthalmology. 2021;128(5):650‑658.
2. American Academy of Ophthalmology. “Epidemiology of Scleritis.” AAO Preferred Practice Pattern, 2022.
3. McCluskey PJ, et al. “Autoimmune disease associations with scleritis.” Rheumatology International. 2020;40(3):513‑522.
4. Mayo Clinic. “Scleritis.” Updated 2023. https://www.mayoclinic.org
5. National Eye Institute (NEI). “Scleritis Fact Sheet.” 2022.
6. World Health Organization. “Global prevalence of autoimmune diseases.” WHO Report, 2022.

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