Learning objectives
- What is Cogan syndrome
- Recognition and treatment
Introduction
- Cogan syndrome is a chronic inflammatory disease of unknown origin
- Can affect young adults
- May cause red eye, ear pain ,vertigo, episcleritis, scleritis, Uveitis, vitritis and choroiditis
Aetiology
- Autoimmune reaction against an unknown common autoantigen in the cornea and inner ear
- Patients can develop an aortitis.
- Keratitis, CNS vasculitis and Deafness and venous sinus thrombosis
Clinical
- Systemic symptoms - fever, headache, joint pain, and myalgia.
- Bilateral sensorineural hearing loss
- Vestibular symptoms
- Inflammatory ocular manifestations - keratitis
- Rarely a severe systemic vasculitis with aortitis.
Investigations
- Bloods: mildly elevated ESR, CRP
- Slit lamp examination examination of the eye
- MRI and MRA brain
Differentials
- Susac's syndrome. Though examination should readily distinguish the two disorders, the physician should consider Susac's syndrome and look for the triad of hearing loss, BRAO, encephalopathy.
Management
- Consider ophthalmology review and systemic corticosteroids for ocular involvement
- Systemic disease - Prednisolone 1 mg/kg po once/day is begun as soon as possible and continued for 2 to 6 mo. Some clinicians add cyclophosphamide, methotrexate, or cyclosporine for recalcitrant cases.
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