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Cogan Syndrome
👁️👂 Cogan Syndrome is a rare autoimmune condition with ocular inflammation, sensorineural hearing loss, and sometimes systemic vasculitis (incl. aortitis). Key exam pearl: distinguish from Susac’s syndrome (triad of BRAOs, encephalopathy, hearing loss).
📖 Introduction
- Chronic inflammatory disease of unknown origin.
- Most often affects young adults.
🧬 Aetiology
- Believed to be an autoimmune reaction against a shared antigen in the cornea and inner ear.
- Can also involve systemic vessels → aortitis and vasculitis.
🔎 Clinical Features
- 🌡️ Systemic: fever, headache, arthralgia, myalgia.
- 👂 Bilateral sensorineural hearing loss, vertigo, tinnitus, vestibular dysfunction.
- 👁️ Ocular: interstitial keratitis (classic), episcleritis, scleritis, uveitis, vitritis, choroiditis.
- ⚠️ Rare: severe systemic vasculitis with aortitis (risk of aneurysm).
🧪 Investigations
- 📊 Bloods: mildly raised ESR/CRP.
- 🔬 Slit-lamp: keratitis, ocular inflammation.
- 🩻 Consider echocardiography or MRI for vasculitis/aortitis if suspected.
🧠 Differential: Susac’s Syndrome → triad of: 1️⃣ Sensorineural hearing loss 2️⃣ Branched retinal artery occlusions (BRAOs) 3️⃣ Encephalopathy
💊 Management
- 👁️ Ophthalmology referral for ocular disease.
- 💊 Systemic corticosteroids: Prednisolone ~1 mg/kg PO daily, taper over 2–6 months.
- ➕ Immunosuppressants (for refractory disease): cyclophosphamide, methotrexate, cyclosporine.
- 🫀 Monitor for systemic vasculitis/aortitis complications.
| The content on this website is provided for educational and informational purposes only to support exam preparation (e.g., MLA, MRCP, USMLE) and learning. This is NOT medical advice, diagnosis, treatment, or professional guidance. It does not replace consultation with a qualified healthcare professional, official guidelines (e.g., NICE, GMC, BNF), or supervised clinical practice. Always verify information with current, authoritative sources. Makindo and its contributors accept no liability for any reliance on this content, including errors, omissions, or any resulting harm, loss, or consequences. By using this site, you agree to these terms. |
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