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A

Cogan syndrome


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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