Jindal Himanshu, Suresh Vinay, Kamaraj Balakrishnan, Jha Mayank, Verma Nikhil, Sharma Awadhesh Kumar, Purushothaman P, Kumar Shubham, Mehta Rachana, Sah Ranjana, Verma Amogh
Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, India.
King George's Medical University, Lucknow, India.
Clin Med Insights Cardiol. 2025 Jun 17;19:11795468251350222. doi: 10.1177/11795468251350222. eCollection 2025.
Takayasu arteritis (TA) is a rare granulomatous vasculitis affecting the aorta and its major branches, while systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder known for vascular and serological involvement. Although both diseases share certain immunopathological pathways, their co-occurrence is exceedingly rare. We report a case of a 44-year-old Indian female with a prior diagnosis of SLE who presented with breathlessness, palpitations, and nodular episcleritis. Cardiovascular evaluation revealed carotid bruit, diminished peripheral pulses, blood pressure discrepancies, and murmurs suggestive of valvular dysfunction. Laboratory investigations demonstrated elevated erythrocyte sedimentation rate, hypochromic microcytic anemia, hypocomplementemia, positive antinuclear and anti-dsDNA antibodies, and proteinuria. Imaging confirmed circumferential thickening of the ascending and descending aorta and occlusive disease in the left carotid system. Based on the 2022 ACR/EULAR classification criteria, a diagnosis of concomitant TA was established. Management included corticosteroids and mycophenolate mofetil, chosen over cyclophosphamide due to borderline renal function and fertility considerations. Despite planning for aortic root replacement, the patient deteriorated and succumbed to complications of severe aortic regurgitation. This case highlights the diagnostic complexities of overlapping autoimmune vasculitides and emphasizes the need for early recognition, rigorous application of classification criteria, and individualized immunosuppressive strategies to optimize outcomes in such rare presentations.
高安动脉炎（TA）是一种罕见的肉芽肿性血管炎，累及主动脉及其主要分支，而系统性红斑狼疮（SLE）是一种多系统自身免疫性疾病，以血管和血清学受累为特征。尽管这两种疾病有某些免疫病理途径相同，但它们同时出现极为罕见。我们报告一例44岁印度女性病例，该患者先前诊断为SLE，现出现呼吸困难、心悸和结节性巩膜炎。心血管评估发现颈动脉杂音、外周脉搏减弱、血压差异以及提示瓣膜功能障碍的杂音。实验室检查显示红细胞沉降率升高、低色素小细胞性贫血、补体血症降低、抗核抗体和抗双链DNA抗体阳性以及蛋白尿。影像学检查证实升主动脉和降主动脉周向增厚以及左颈动脉系统闭塞性疾病。根据2022年美国风湿病学会/欧洲抗风湿病联盟分类标准，确诊为合并TA。治疗包括使用皮质类固醇和霉酚酸酯，由于肾功能临界状态和生育方面的考虑，选择了霉酚酸酯而非环磷酰胺。尽管计划进行主动脉根部置换，但患者病情恶化，死于严重主动脉瓣关闭不全的并发症。该病例突出了重叠性自身免疫性血管炎的诊断复杂性，并强调了早期识别、严格应用分类标准以及个体化免疫抑制策略对于优化此类罕见病例治疗结果的必要性。
