Scleral Manifestations of Systemic Disease

Scleral Manifestations of Systemic Disease

Scleral Manifestations of Systemic Disease

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CTDs & Sclera - Joint & Eye Woes

Connective tissue diseases (CTDs) frequently manifest in the sclera, causing inflammation (scleritis, episcleritis) and structural changes. Systemic workup crucial.

  • Major CTD Associations:
    • Rheumatoid Arthritis (RA): Most common systemic cause of scleritis, esp. necrotizing.
    • Granulomatosis with Polyangiitis (GPA): Necrotizing scleritis, often with Peripheral Ulcerative Keratitis (PUK).
    • Systemic Lupus Erythematosus (SLE): Episcleritis more frequent than scleritis.
    • Relapsing Polychondritis: Episcleritis, scleritis, chondritis (auricular/nasal).
    • Ankylosing Spondylitis, Inflammatory Bowel Disease (IBD).
  • Types of Scleral Involvement:
    • Episcleritis: Superficial, sectoral/diffuse redness, mild discomfort. Often self-limiting.
    • Scleritis: Deep, violaceous hue. Severe, boring pain (radiates, nocturnal).
      • Anterior: Diffuse, Nodular, Necrotizing (high visual loss risk).
        • Necrotizing with inflammation: Most severe.
        • Necrotizing without inflammation (Scleromalacia Perforans): In long-standing RA, perforation risk. 📌 "Painless perforation".
      • Posterior: Pain, ↓vision, proptosis, choroidal folds.

How Arthritis Affects the Eye

⭐ Scleromalacia perforans, a necrotizing scleritis without significant inflammation, is classically seen in women with long-standing, seropositive Rheumatoid Arthritis.

Vasculitis & Infections - Vessel & Bug Alerts

  • Vasculitis-Associated Scleritis:
    • Granulomatosis with Polyangiitis (GPA): Necrotizing scleritis common, often bilateral; c-ANCA/PR3-ANCA positive.
    • Polyarteritis Nodosa (PAN): Necrotizing scleritis; Hepatitis B association.
    • Relapsing Polychondritis: Affects cartilage (ears, nose) & sclera; varied scleritis types.
    • Behçet's Disease: Scleritis can occur, often with uveitis.
    • SLE: Less common, typically diffuse/nodular scleritis.
  • Infectious Scleritis: Intense pain, redness, photophobia; purulent discharge signals infection.
    • Bacterial:
      • Pseudomonas aeruginosa: 📌 Post-Surgical Problem. Most common after surgery/trauma; aggressive.
      • Staphylococcus aureus, Streptococcus pneumoniae.
      • Tuberculosis (TB): Nodular or necrotizing; requires systemic Anti-Tubercular Therapy (ATT).
    • Viral:
      • Herpes Zoster Ophthalmicus (HZO): Scleritis, often with keratitis/uveitis. Hutchinson's sign (tip of nose vesicles) is key.
      • Herpes Simplex Virus (HSV).
    • Fungal: Aspergillus, Candida. Immunocompromised or post-trauma (e.g., vegetative injury).
    • Protozoal: Toxoplasma, Acanthamoeba (rare for scleritis).

⭐ Infectious scleritis, especially bacterial (e.g., Pseudomonas), can rapidly progress to scleral perforation if not treated aggressively with appropriate antimicrobials.

  • Alkaptonuria (Ochronosis):
    • Bluish-black scleral pigmentation (near muscle insertions). Homogentisic acid.
  • Gout:
    • Scleral/episcleral urate tophi; recurrent inflammation.
  • Porphyria (e.g., PCT):
    • Scleral thinning, photosensitivity; risk of scleromalacia.
  • Osteogenesis Imperfecta (OI):
    • Blue sclera (thin sclera, uvea visible). 📌 Brittle Bones, Blue Sclera. Blue sclera in Osteogenesis Imperfecta
  • Ehlers-Danlos Syndrome (EDS):
    • Blue sclera, scleral fragility, ↑ globe rupture risk.
  • Scleral Icterus (Jaundice):
    • Yellow sclera (↑ bilirubin).

    ⭐ Often first sign of jaundice; visible if serum bilirubin > 2-3 mg/dL.

  • Other Rare Links:
    • Cystinosis: Corneal crystals primary; scleral deposits possible.
    • Mucopolysaccharidoses (MPS): Scleral thickening (some types).
    • Ligneous Conjunctivitis: May extend to sclera, cornea (woody pseudomembranes).

High‑Yield Points - ⚡ Biggest Takeaways

  • Rheumatoid Arthritis is the most common systemic association with scleritis, especially necrotizing scleritis.
  • Ankylosing Spondylitis often presents with anterior uveitis and scleritis.
  • Wegener's Granulomatosis (GPA) can cause necrotizing scleritis and peripheral ulcerative keratitis (PUK).
  • Inflammatory Bowel Disease (IBD), like Crohn's and Ulcerative Colitis, is linked to episcleritis and scleritis.
  • Sarcoidosis may manifest as granulomatous scleritis or nodular episcleritis.
  • Blue sclera is characteristic of Osteogenesis Imperfecta; also seen in Ehlers-Danlos syndrome and Marfan syndrome.
  • Scleromalacia perforans is a severe, painless necrotizing scleritis often seen in long-standing Rheumatoid Arthritis women.

Practice Questions: Scleral Manifestations of Systemic Disease

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What are the extra-articular manifestations of RA?

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Flashcards: Scleral Manifestations of Systemic Disease

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Surgically induced _____ (SINS) may occur following any surgery involving the creation of a scleral wound

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Surgically induced _____ (SINS) may occur following any surgery involving the creation of a scleral wound

necrotizing scleritis

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