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A Mekinian1, J Pouchot2, T Zenone3

  • 1Sorbonne Université, AP-HP, Hôpital Saint-Antoine, service de médecine interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), 75012 Paris, France.

La Revue De Medecine Interne
|August 11, 2020
PubMed
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Cogan's syndrome involves eye and ear inflammation, with typical cases showing keratitis and Meniere-like symptoms. Atypical presentations and rare vascular issues require careful management, often with steroids.

Area of Science:

  • Ophthalmology
  • Rheumatology
  • Otolaryngology

Background:

  • Cogan's syndrome is a rare inflammatory condition affecting ocular and auditory systems.
  • It presents typically with interstitial keratitis and Meniere-like audio-vestibular dysfunction.
  • Atypical forms lack keratitis or exhibit delayed/different audio-vestibular or ocular symptoms.

Purpose of the Study:

  • To define the clinical spectrum and diagnostic criteria for Cogan's syndrome.
  • To highlight the potential for systemic vascular involvement, particularly large-vessel vasculitis.
  • To outline prognostic factors and therapeutic strategies.

Main Methods:

  • Review of clinical presentations and diagnostic features of Cogan's syndrome.
  • Analysis of disease progression, complications, and treatment outcomes.
Keywords:
Cogan's syndromeSyndrome de CoganVasculariteVasculitis

Related Experiment Videos

  • Classification of typical versus atypical Cogan's syndrome based on key manifestations.

    • Main Results:

    • Typical Cogan's syndrome involves interstitial keratitis and audio-vestibular impairment within 2 years.
    • Atypical Cogan's syndrome includes varied ocular/auditory signs, delayed onset, or systemic vasculitis (e.g., thoracic aorta).
    • Prognosis is primarily influenced by audio-vestibular impairment and risk of deafness; vascular complications are rare.

    Conclusions:

    • Cogan's syndrome requires a high index of suspicion due to its varied presentations.
    • Early diagnosis and treatment, often with glucocorticoids, are crucial to prevent irreversible hearing loss.
    • Systemic evaluation for vasculitis is warranted in suspected cases, guiding further immunosuppressive or targeted therapy.