Vertigo Center, Cheikh Khalifa International University HospitalMohammed VI University of Health Sciences, Casablanca, Morocco.
Internal Medicine, Cheikh Khalifa International University Hospital, Mohammed VI University of Health Sciences, Casablanca, Morocco.
J Med Case Rep. 2024 Nov 14;18(1):551. doi: 10.1186/s13256-024-04904-6.
Cogan syndrome is a rare autoimmune systemic vasculitis presenting with interstitial keratitis and audiovestibular symptoms. The atypical form, characterized by more extensive ocular lesions with audiovestibular symptoms appearing with a longer delay and more frequent systemic features, is usually underdiagnosed, delaying treatment.
We report the case of a 30-year-old Mediterranean female who presented recurrent left red and painful eye. The evolution in flare-ups during 3 years, associated with a nonspecific biological inflammatory response, motivated ocular biopsies demonstrating diffuse inflammatory changes from the cornea to the sclera. Since Morocco is an endemic region for tuberculosis, the laboratory tests were expanded to identify the latent forms based on the tuberculin skin test and QuantiFERON TB Gold in tube assay, both positive for Mycobacterium tuberculosis infection. Thus, antibacillary chemotherapy was started for 6 months, with reappearance of the symptoms at the end of treatment. Three years later, the patient presented isolated and fluctuating audiovestibular Ménière-like symptoms, with progressive sensorineural hearing loss. The nonstabilization under usual medical treatment along with her diffuse inflammatory ocular lesions led to the diagnosis of atypical Cogan syndrome. The patient received long-term corticosteroid with notable improvement.
Cogan syndrome is a rare autoimmune disease that should be considered when faced with ocular and audiovestibular manifestations, even in its atypical form, to provide early and adequate treatment, which is the main prognosis factor in the control of irreversible lesions. Thus, interdisciplinary collaboration is fundamental along with screening for other infectious and systemic disorders that should include tuberculosis.
Cogan 综合征是一种罕见的自身免疫性系统性血管炎，表现为间质性角膜炎和听觉-前庭症状。不典型形式的特点是更广泛的眼部病变，听觉-前庭症状出现的时间更长，且更常出现全身症状，通常被误诊，导致治疗延迟。
我们报告了一位 30 岁的地中海女性患者，她反复出现左眼发红和疼痛。3 年来，疾病反复发作，伴有非特异性生物炎症反应，促使进行眼部活检，结果显示从角膜到巩膜均有弥漫性炎症改变。由于摩洛哥是结核病的流行地区，因此实验室检查扩大到基于结核菌素皮肤试验和 QuantiFERON TB Gold 管内检测来识别潜伏形式，两者均为结核分枝杆菌感染阳性。因此，开始进行抗结核化学疗法治疗 6 个月，治疗结束时症状再次出现。3 年后，患者出现孤立性和波动性听觉-前庭梅尼埃样症状，伴有进行性感觉神经性听力损失。由于常规药物治疗未能稳定病情，且患者存在弥漫性炎症性眼部病变，因此诊断为不典型 Cogan 综合征。患者接受了长期皮质类固醇治疗，病情显著改善。
Cogan 综合征是一种罕见的自身免疫性疾病，当出现眼部和听觉-前庭表现时，即使是不典型形式，也应考虑到该病，以便早期和充分治疗，这是控制不可逆病变的主要预后因素。因此，跨学科合作是基础，同时还应筛查其他感染和系统性疾病，包括结核病。
