Necrotizing Scleritis

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Definition & Causes - Scleral Siege

  • Definition: Severe, destructive scleral inflammation with scleral thinning, necrosis; risk of perforation. Intense, boring pain is hallmark.
  • Types:
    • Necrotizing with inflammation: Acutely painful.
    • Necrotizing without inflammation (Scleromalacia Perforans): Often painless; typically in longstanding Rheumatoid Arthritis.
  • Primary Associations:
    • Systemic Autoimmune (most common, ≈50-60%):
      • Rheumatoid Arthritis (RA)
      • Granulomatosis with Polyangiitis (GPA)
      • Systemic Lupus Erythematosus (SLE)
      • Relapsing Polychondritis
    • Infectious (rare): Herpes Zoster, TB, Syphilis.
    • Surgically-induced: Post-operative. Necrotizing Scleritis Progression

⭐ Most common association of necrotizing scleritis is with systemic autoimmune diseases, particularly Rheumatoid Arthritis.

Clinical Picture - Eye on Fire

  • Pain: Severe, boring, deep ocular pain.
    • Radiates: Temple, jaw, sinuses.
    • Nocturnal: Characteristically awakens patient at night.
  • Redness: Intense, violaceous, "beefy-red" diffuse or nodular congestion; non-blanching with topical phenylephrine.
  • Photophobia, lacrimation.
  • ↓ Vision: Often progressive, can be severe.
  • Scleral Appearance:
    • Thinning: Sclera may appear translucent, revealing underlying bluish uvea.
    • Avascular patches: Indicative of necrosis.
    • High risk of scleral melt and perforation.
  • Commonly associated with systemic autoimmune diseases (e.g., Rheumatoid Arthritis, GPA, SLE). Necrotizing Scleritis with Severe Inflammation

⭐ The hallmark symptom is severe, boring ocular pain that may radiate to the temple, jaw, or sinuses, and characteristically awakens the patient at night.

Diagnosis & Workup - Unmasking the Culprit

Diagnostic Testing for Scleritis

  • Slit-lamp Exam: Confirms scleral thinning, edema, avascular patches, and inflammation. Note extreme pain.
  • Imaging:
    • B-scan Ultrasonography: Assesses posterior involvement, scleral thickness.
    • Ultrasound Biomicroscopy (UBM): Details anterior scleral changes.
  • Systemic Evaluation (Crucial):
    • Inflammatory markers: ESR, CRP.
    • Autoantibodies: RF, anti-CCP, ANCA, ANA.
    • Chest X-ray; consider infectious screen (e.g., syphilis, TB).

⭐ B-scan ultrasonography is invaluable for assessing posterior scleritis, scleral thickness, and associated fluid or choroidal detachment.

Complications & Outlook - Danger Zone

  • Scleral thinning → melt → perforation (esp. scleromalacia perforans)
  • Uveitis (anterior/posterior), PUK, glaucoma, cataract
  • Retinal detachment, choroidal effusion, optic neuropathy
  • Severe, irreversible vision loss; globe deformity, phthisis bulbi
  • Outlook: Guarded. Requires prompt, aggressive immunosuppression.
  • High morbidity; mortality risk if systemic vasculitis untreated.

⭐ Scleral perforation is a dreaded complication, especially in the 'scleromalacia perforans' variant, leading to potential globe rupture and severe vision loss. Necrotizing Scleritis with Scleral Perforation A

Treatment Strategy - Quelling the Flames

  • Primary Goal: Arrest inflammation, alleviate pain, prevent scleral melt & vision loss.
  • Cornerstone: Prompt, aggressive systemic immunosuppression.

    ⭐ Aggressive systemic immunosuppression (e.g., corticosteroids combined with cyclophosphamide or biologic agents) is the mainstay of treatment for necrotizing scleritis to prevent visual loss and systemic complications.

  • Initial Regimen:
    • Systemic Corticosteroids: High-dose (e.g., IV Methylprednisolone pulse, then oral Prednisolone 1-1.5 mg/kg/day).
    • PLUS Immunosuppressive agent: Cyclophosphamide (IV or oral), Methotrexate, Azathioprine, Mycophenolate.
  • Refractory/Severe Disease:
    • Biologic agents (e.g., Infliximab, Adalimumab, Rituximab).
  • ⚠️ Avoid: Topical steroids alone (ineffective, risk masking perforation).
  • Surgery: Reserved for complications (e.g., tectonic scleral patch graft for perforation).

High‑Yield Points - ⚡ Biggest Takeaways

  • Most severe and destructive scleritis, often causing significant visual loss.
  • Strong association with systemic autoimmune diseases (e.g., Rheumatoid Arthritis, ANCA-vasculitis).
  • Intense, boring, constant pain, often worse at night and radiating.
  • High risk of scleral thinning, staphyloma, and globe perforation.
  • Requires prompt, aggressive systemic immunosuppression (corticosteroids, cytotoxic agents).
  • Scleromalacia perforans: a specific painless necrotizing variant in long-standing Rheumatoid Arthritis.

Practice Questions: Necrotizing Scleritis

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Uveoparotid fever is seen in:

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What part of eye is affected by nevus of Ota?_____

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What part of eye is affected by nevus of Ota?_____

Sclera

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