Autoimmune Disorders

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Sjögren's & KCS - Desert Eyes Drama

  • Autoimmune disorder targeting exocrine glands (lacrimal, salivary) → Keratoconjunctivitis Sicca (KCS) & xerostomia.
  • Primary Sjögren's: Sicca symptoms alone (dry eyes, dry mouth).
  • Secondary Sjögren's: Associated with other autoimmune diseases (e.g., RA, SLE).
  • KCS Symptoms: Dryness, grittiness, burning, foreign body sensation, photophobia, blurred vision.
    • Signs: ↓ Tear film, conjunctival injection, corneal erosions (SPKs), filaments, mucus strands.
  • Diagnosis: Key tests include:
    • Schirmer's test: Measures tear production (<5mm in 5 min is significant).
    • Tear Break-Up Time (TBUT): Assesses tear film stability (<10s is abnormal).
    • Ocular surface staining: Rose Bengal, Lissamine Green highlight damaged cells.
    • Serology: Anti-Ro (SSA) & Anti-La (SSB) antibodies.
  • Management: Artificial tears, lubricating ointments, punctal occlusion, topical cyclosporine A, lifitegrast.

Rose Bengal staining in Keratoconjunctivitis Sicca

⭐ Schirmer's test I (without topical anesthesia) value of <5mm wetting in 5 minutes is a key diagnostic criterion for aqueous-deficient dry eye, often seen in Sjögren's syndrome.

Rheumatoid Arthritis - Joint & Eye Plight

Necrotizing scleritis and peripheral ulcerative keratitis

  • Keratoconjunctivitis Sicca (KCS): Most common ocular manifestation; results in dry eyes.
  • Scleritis: Severe, deep ocular pain. Anterior (diffuse/nodular) most common.
    • Necrotizing scleritis: Most severe form; can lead to scleromalacia perforans (painless blue patches, perforation risk).
  • Episcleritis: More common than scleritis; often recurrent, self-limiting.
  • Peripheral Ulcerative Keratitis (PUK): Crescent-shaped stromal inflammation and thinning at limbus; "corneal melt". High perforation risk.
  • Uveitis: Less frequent compared to spondyloarthropathies.
  • 📌 Mnemonic: Sicca, Scleritis, PUK for RA.

⭐ Necrotizing scleritis in RA is a severe, sight-threatening condition that can lead to scleromalacia perforans, characterized by areas of scleral thinning and potential globe rupture.

Spondyloarthropathies & Uveitis - HLA-B27's Eye Ire

  • Spondyloarthropathies (SpA): Inflammatory diseases (📌 PAIR: Psoriatic arthritis, Ankylosing spondylitis, IBD-arthritis, Reactive arthritis).
  • Strong HLA-B27 link.
  • Ocular Hallmark: Acute Anterior Uveitis (AAU).
    • Most common extra-articular manifestation, esp. in Ankylosing Spondylitis (AS).
    • Sudden: pain, redness, photophobia, ↓vision.
    • Signs: Ciliary flush, keratic precipitates (KPs), AC cells/flare, hypopyon (sometimes).
    • Typically non-granulomatous, recurrent, unilateral; may alternate eyes. Clinical signs of acute anterior uveitis
  • Reactive Arthritis (Reiter's): Triad "Can't see, can't pee, can't climb a tree" (uveitis/conjunctivitis, urethritis, arthritis).
  • Complications: Posterior synechiae, cataract, secondary glaucoma, cystoid macular edema.

⭐ In Ankylosing Spondylitis, AAU occurs in 25-40% of patients.

Giant Cell Arteritis - Vision's Urgent Fright

  • Vasculitis of large/medium arteries; age >50 yrs (📌 50/50 rule: Age >50, ESR >50).
  • Symptoms: New headache, jaw claudication, scalp tenderness, fever, PMR.
  • Ocular EMERGENCY:
    • AAION: Sudden, painless, severe vision loss.
    • Amaurosis fugax, diplopia.
  • Signs: Tender, pulseless temporal artery. AAION: Chalky-white swollen disc, APD.
  • Dx: ↑ ESR (>50 mm/hr), ↑ CRP. Temporal Artery Biopsy (TAB) definitive. CDUS: "halo sign".
  • Rx: URGENT! Start steroids BEFORE biopsy.
    • IV Methylprednisolone (1g/day x 3d) for vision loss.
    • Oral Prednisolone (1mg/kg/day).

⭐ Untreated GCA can lead to bilateral blindness in days to weeks; prompt high-dose steroids are vision-saving.

High‑Yield Points - ⚡ Biggest Takeaways

  • Sjögren's Syndrome: Keratoconjunctivitis Sicca (KCS) is hallmark; positive Schirmer's/Rose Bengal staining.
  • Rheumatoid Arthritis: Scleritis (especially necrotizing), episcleritis, KCS, and corneal melt (PUK).
  • SLE: Lupus retinopathy (cotton wool spots, hemorrhages), optic neuropathy, and KCS.
  • Ankylosing Spondylitis: Acute anterior uveitis (recurrent, unilateral, often HLA-B27 positive).
  • Sarcoidosis: Granulomatous uveitis ("candle wax drippings", mutton-fat KPs), panuveitis, lacrimal gland involvement.
  • Behçet's Disease: Recurrent hypopyon uveitis, retinal vasculitis, and occlusive vasculopathy.
  • Giant Cell Arteritis: AION is a blinding risk; amaurosis fugax, ↑ESR, immediate high-dose steroids.

Practice Questions: Autoimmune Disorders

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An elderly male presents with pain in his shoulders and hands. ESR is 105 mm/L. History includes transient blindness and unilateral headache.

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Flashcards: Autoimmune Disorders

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_____ nodule seen at the anterior surface of the iris in anterior uveitis

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_____ nodule seen at the anterior surface of the iris in anterior uveitis

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