Cogans syndrom - när flera av våra sinnen spökar. Ögon och öron påverkas
Sammanfattning
Cogans syndrome is a rare systemic inflammatory disease characterized by a combination of audiovestibular and ocular symptoms. In
some cases, systemic complications occur with vascular inflammation. Aortitis and large vessel vasculitis are the most common forms, but
medium-sized and small vessel involvement has also been described. The autoimmune reaction can lead to blindness, deafness and in worst
case death, if these patients remain untreated or if treatment is delayed. There is no specific blood test or imaging method available and the
diagnosis is clinical. It should be suspected in patients presenting with both inflammatory eye symptoms and audiovestibular dysfunction, when
more common autoimmune and infectious diseases have been excluded. The treatment consists of high dose systemic steroids, topical
steroids for the affected eye and in some cases addition of immunosuppressive drugs. Treatment is based on the severity of the symptoms and
how well the patient responds to initial systemic corticosteroids. Here we present a case of suspected Cogan’s syndrome where diagnosis was
considered after exclusion of other possible autoimmune and infectious diseases.