Hedin Erika, Lyckberg Helge, Carlsson Per-Inge
underläkare, Centralsjukhuset Karlstad - öron-, näsa- och halskliniken Karlstad, Sweden Centralsjukhuset Karlstad - ÖNH-kliniken Karlstad, Sweden.
överläkare, Centralsjukhuset Karlstad - öron-, näsa- och halskliniken Karlstad, Sweden överläkare, Centralsjukhuset Karlstad - öron-, näsa- och halskliniken Karlstad, Sweden.
Lakartidningen. 2016 Feb 23;113:DUWT.
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.
科根综合征是一种罕见的全身性炎症性疾病，其特征为听觉前庭和眼部症状同时出现。在某些情况下，会出现伴有血管炎症的全身并发症。主动脉炎和大血管血管炎是最常见的形式，但也有中、小血管受累的报道。如果这些患者未得到治疗或治疗延迟，自身免疫反应可能导致失明、失聪，最严重的情况下会导致死亡。目前尚无特定的血液检查或影像学方法，诊断依靠临床症状。当排除了更常见的自身免疫性和感染性疾病后，对于同时出现炎症性眼部症状和听觉前庭功能障碍的患者应怀疑患有该病。治疗包括大剂量全身性类固醇、患眼局部使用类固醇，某些情况下还需加用免疫抑制药物。治疗方案依据症状的严重程度以及患者对初始全身性皮质类固醇的反应情况而定。在此，我们报告一例疑似科根综合征的病例，该病例在排除其他可能的自身免疫性和感染性疾病后得以确诊。
