Lu Ci, Lv Panpan, Zhu Xiaoying, Han Yongmei
Department of Rheumatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China.
Department of Ultrasound, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, People's Republic of China.
J Inflamm Res. 2024 Mar 20;17:1839-1843. doi: 10.2147/JIR.S453071. eCollection 2024.
Cogan's syndrome (CS) is a rare chronic inflammatory disease, characterized by interstitial keratitis and vestibular auditory dysfunction. Hypertrophic pachymeningitis (HP) is a rare chronic aseptic inflammatory disease of the central nervous system. This article reports a patient with CS coexisting with HP. The patient was a 66-year-old male with fever, headache, red eyes, hearing loss, and significantly elevated inflammatory markers. Cerebrospinal fluid examination, blood culture, and tests for autoantibodies such as antinuclear antibodies were negative. Pure tone audiology (PTA) indicated bilateral sensorineural deafness. Both Positron emission tomography-computed tomography (PET/CT) and vascular color Doppler ultrasound suggest the presence of vasculitis. Considering Cogan's syndrome, the patient received 40 mg of methylprednisolone intravenously once daily. The brain's magnetic resonance imaging (MRI) revealed slightly thickened and enhanced dura mater, suggesting HP. The dose of methylprednisolone was increased to 40 mg intravenously every 8 hours, leading to the patient's improved symptoms and decreased inflammatory markers. Both CS and HP are rare chronic inflammatory diseases, and their coexistence is even rarer, with only two reported cases in literature up to date. The coexistence of CS and HP should be considered when the CS patients with headaches do not respond well to treatment.
科根综合征（CS）是一种罕见的慢性炎症性疾病，其特征为间质性角膜炎和前庭听觉功能障碍。肥厚性硬脑膜炎（HP）是一种罕见的中枢神经系统慢性无菌性炎症性疾病。本文报告了一名CS合并HP的患者。该患者为66岁男性，有发热、头痛、眼红、听力丧失，炎症标志物显著升高。脑脊液检查、血培养以及抗核抗体等自身抗体检测均为阴性。纯音听力测定（PTA）显示双侧感音神经性耳聋。正电子发射断层扫描-计算机断层扫描（PET/CT）和血管彩色多普勒超声均提示存在血管炎。考虑为科根综合征，患者接受每日一次静脉注射40 mg甲泼尼龙治疗。脑部磁共振成像（MRI）显示硬脑膜轻度增厚并强化，提示HP。甲泼尼龙剂量增加至每8小时静脉注射40 mg，患者症状改善，炎症标志物下降。CS和HP均为罕见的慢性炎症性疾病，二者并存更为罕见，迄今为止文献中仅报道过两例。当CS患者头痛对治疗反应不佳时，应考虑CS与HP并存的情况。
