The Second Department of Internal Medicine, University of Toyama, 2630 Sugitani, Toyama, 930-0194, Japan.
Department of Diagnostic Pathology, University of Toyama, Toyama, Japan.
CEN Case Rep. 2024 Jun;13(3):154-160. doi: 10.1007/s13730-023-00819-1. Epub 2023 Sep 22.
Takayasu arteritis is a rare, chronic, and large-vessel vasculitis involving the aorta and its branches in a complex autoimmune reaction. Takayasu arteritis sometimes complicates aortic regurgitation and chronic kidney disease, but rarely accompanies nephrotic syndrome. We had a patient with Takayasu arteritis and concomitant aortic regurgitation. She had nephrotic syndrome that was refractory to immunosuppressive therapy but was promptly improved after surgical aortic valve replacement. In her kidney biopsy, glomeruli had mild mesangial proliferative changes without immune complex deposition. Her proteinuria remained negative until the recurrence of aortic regurgitation due to perivalvular leakage. Seventeen years after the surgery, she died suddenly. In her kidney autopsy, the arteriolar showed severe hyalinosis and the glomerulus showed mesangial proliferative changes with segmental mesangiolysis. Severe aortic regurgitation may have altered renal hemodynamics and caused glomerular lesions, resulting in nephrotic syndrome. We should be aware of the rare but critical comorbidity of nephrotic syndrome in patients with Takayasu arteritis and concomitant aortic regurgitation.
Takayasu 动脉炎是一种罕见的、慢性的、累及主动脉及其分支的大血管炎，是一种复杂的自身免疫反应。Takayasu 动脉炎有时会并发主动脉瓣反流和慢性肾脏病，但很少伴有肾病综合征。我们有一位 Takayasu 动脉炎合并主动脉瓣反流的患者。她患有肾病综合征，对免疫抑制治疗无反应，但在接受主动脉瓣置换手术后迅速改善。她的肾脏活检显示肾小球有轻度系膜增生性改变，无免疫复合物沉积。她的蛋白尿一直呈阴性，直到因瓣周漏导致主动脉瓣反流复发。术后 17 年，她突然去世。她的肾脏尸检显示，小动脉严重玻璃样变，肾小球系膜增生性改变伴节段性系膜溶解。严重的主动脉瓣反流可能改变了肾脏的血液动力学，导致肾小球病变，从而导致肾病综合征。我们应该意识到，Takayasu 动脉炎合并主动脉瓣反流的患者中，肾病综合征虽然罕见，但却很关键。
