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Cogan syndrome

Learning objectives

  • What is Cogan syndrome
  • Recognition and treatment

Introduction

Cogan syndrome is a chronic inflammatory disease of unknown origin that may cause stroke and affects young adults which typically causes 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 and 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 1) hearing loss, 2) branched retinal artery occlusion and 3) 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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