What is Cogans syndrome?
Cogans syndrome has a median age of onset of 22 years (range, 5–63 years) and no gender or racial predilection. Up to 33% have an antecedent viral illness before symptom onset. It has the following manifestations:
• Major manifestations: occur concurrently or within 4 months in 75% of patients.
• Ocular disease (red, painful, photophobic eyes): typical lesion is nonsyphilitis interstitial keratitis. Can have scleritis, episcleritis, uveitis, or chorioretinitis.
• Audiovestibular symptoms: rapid onset sensorineural hearing loss (often bilateral) and vestibular dysfunction (vertigo and ataxia). Can develop cochlear hydrops.
• Other manifestations: tend to occur in patients who are at risk for widespread vasculitis.
• Constitutional symptoms (50%): fever, weight loss, adenopathy, hepatosplenomegaly, arthritis, others.
• Vasculitis: aortitis with aortic insufficiency/aneurysm (12%), coronary vasculitis, aortic/mitral valvulitis, purpura, gangrene.
• Laboratory abnormalities: anemia, leukocytosis, thrombocytosis, elevated ESR and CRP. Rarely antimyeloperoxidase (perinuclear antineutrophil cytoplasmic antibodies) and anti-Hsp-70 antibodies.
