Sierra Laura Villegas, Binzenhöfer Leonhard, Schulze-Koops Hendrik, Lackermair Korbinian, Massberg Steffen, Lüsebrink Enzo
Medizinische Klinik und Poliklinik I, Klinikum der Universität München, Munich, Germany.
DZHK (German Center for Cardiovascular Research), Partner Site Munich Heart Alliance, Munich, Germany.
Eur Heart J Case Rep. 2023 May 31;7(6):ytad263. doi: 10.1093/ehjcr/ytad263. eCollection 2023 Jun.
Takayasu arteritis (TA) is a rare large-vessel vasculitis primarily affecting the aorta and its proximal branches. The manifestation of TA is variable, ranging from asymptomatic cases to severe organ dysfunction secondary to vascular damage, which often delays diagnosis.
Here, we present a 37-year-old male patient suffering from visual impairment and malignant hypertension. Emergency fundoscopy showed large left subretinal bleeding and bilateral signs of hypertensive retinopathy. Echocardiographic and magnetic resonance imaging showed mildly reduced left ventricular ejection fraction and signs of hypertensive cardiomyopathy. Evaluation for secondary causes of arterial hypertension did not reveal an underlying disease, and the patient was discharged with optimal medical therapy. He was re-admitted after 11 days with fever of unknown origin, fatigue, and elevated inflammatory markers. The diagnosis of TA was finally established using F-fluorodeoxyglucose positron emission computed tomography scan and sonography of carotid and subclavian arteries. Anti-inflammatory combination therapy for active, severe TA with ophthalmologic involvement was initiated using high-dose glucocorticoids and the tumour necrosis factor alpha inhibitor adalimumab to minimize drug-related risks. The patient was scheduled for multidisciplinary follow-up appointments, including specialist consultation in rheumatology, angiology, cardiology, diabetology, and ophthalmology.
This case highlights the diversity of initial symptoms, the challenges of TA diagnosis, and the importance of comprehensive evaluation for rare secondary causes of arterial hypertension. Individualized acute and long-term care necessitates multidisciplinary management of immunosuppressive therapy, secondary organ involvement, and concomitant diseases.
大动脉炎(TA)是一种罕见的大血管血管炎,主要累及主动脉及其近端分支。TA的表现多种多样,从无症状病例到因血管损伤继发的严重器官功能障碍,这常常导致诊断延迟。
在此,我们报告一名37岁男性患者,患有视力障碍和恶性高血压。紧急眼底检查显示左侧视网膜下大量出血以及双侧高血压视网膜病变体征。超声心动图和磁共振成像显示左心室射血分数轻度降低以及高血压性心肌病体征。对动脉高血压的继发原因进行评估未发现潜在疾病,患者接受最佳药物治疗后出院。11天后,他因不明原因发热、疲劳和炎症标志物升高再次入院。最终通过F-氟脱氧葡萄糖正电子发射计算机断层扫描以及颈动脉和锁骨下动脉超声检查确诊为TA。对于累及眼部的活动期重症TA,开始使用高剂量糖皮质激素和肿瘤坏死因子α抑制剂阿达木单抗进行抗炎联合治疗,以将药物相关风险降至最低。该患者被安排进行多学科随访预约,包括风湿病学、血管病学、心脏病学、糖尿病学和眼科的专家会诊。
本病例突出了初始症状的多样性、TA诊断的挑战以及对罕见的动脉高血压继发原因进行全面评估的重要性。个体化的急性和长期护理需要对免疫抑制治疗、继发器官受累和合并疾病进行多学科管理。