Table 1 Diagnostic criteria and possible differential diagnoses of Cogan syndrome
Mandatory criteria | Clinical manifestations |
|---|---|
Auditory-vestibular involvement | Rapidly progressing sensorineural hearing loss |
Inflammatory ocular disease | Interstitial keratitis, iridocyclitis, conjunctivitis, episcleritis, anterior and posterior uveitis, retinal vasculitis, acute angle closure glaucoma, papillitis, central vein occlusion, vasculitic optic neuropathy, papilledema |
Exclusion of alternative causes | Syphilis, tuberculosis, chlamydia, sarcoidosis, polyarteritis nodosa, ANCA associated vasculitides (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis), Behcet´s disease, Takayasu arteritis, Vogt-Koyanagi-Harada syndrome, Susac syndrome, connective tissue disorders |
Possible additional criteria | |
Nonspecific systemic symptoms | Fever, weight loss, fatigue, lymphadenopathy |
Neurological | Headache, hemiparesis or hemiplegia, aphasia, Encephalitis, peripheral neuropathy |
Vestibular | Vertigo, dizziness, tinnitus, ataxia |
Cardiovascular | Aortitis, vasculitis of other vessels including coronary, renal or mesenteric arteries, peri/myocarditis |
Musculoskeletal | Arthralgias/arthritis, myalgias/myositis |
Gastrointestinal | Diarrhoea, rectal bleeding or melena, abdominal pain, peptic or colonic ulcerations, hepatosplenomegaly |
Laboratory | Increased systemic inflammatory markers |