間質性角膜炎やブドウ膜炎などの眼科的症状、感音性難聴や耳鳴、回転性めまいなどの耳鼻科的症状を伴う血管炎である。

大動脈から中型血管、小型血管まで種々のサイズの動脈が障害される。

大動脈瘤の形成が認められる場合や、大動脈弁や僧帽弁が炎症性に侵される場合もある。

Cogan's syndrome is a rare autoimmune vasculitis, with unknown pathogenesis.まれな自己免疫性の血管炎である

Infection was thought to have played a role in the pathogenesis of the disease, but now the autoimmunity hypothesis is considered more likely to be true. 感染に伴うものと一時は考えられていたが今は自己免疫性と考えるのが妥当と思われる

Cogan's syndrome is characterized by ocular and audiovestibular symptoms similar to those of Meniere's syndrome. 眼窩内や内耳性の病変が主体でメニエル病に似ている

Approximately 70% of the patients have systemic disease, of which vasculitis is considered the pathological mechanism.患者の70％が全身性の病態である

Corticosteroids are the first line of treatment; multiple immunosuppressive drugs were also used with varying degrees of success. The novelty in the treatment of the disease is tumor necrosis factor (TNF)-alpha-blockers, but more studies are necessary to establish their efficacy.ステロイドで治療を始めるが免疫抑制剤やTNFα阻害剤も検討の余地がある

Cogan's syndrome is a rare chronic vasculitis, characterized in its typical form by progressive sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. 進行性で聴覚神経が両側で侵される　非梅毒性の角膜炎、前庭神経症状が出る

Only a few cases have been reported in children, most of whom have been diagnosed with the typical form. Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and infliximab), rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years.