Nephrotic syndrome induced by aortic regurgitation with Takayasu arteritis: an autopsy case with long-term clinical follow-up.

IF 1 Q4 UROLOGY & NEPHROLOGY CEN Case Reports Pub Date : 2024-06-01 Epub Date: 2023-09-22 DOI:10.1007/s13730-023-00819-1
Kota Kakeshita, Teruhiko Imamura, Akira Noguchi, Sayaka Murai, Hayato Fujioka, Hidenori Yamazaki, Tsutomu Koike, Koichiro Kinugawa
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Abstract

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.

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主动脉瓣反流合并大动脉炎引起的肾病综合征:一例尸检病例及长期临床随访。
大动脉炎是一种罕见的慢性大血管血管炎,涉及主动脉及其分支,是一种复杂的自身免疫反应。大动脉炎有时会使主动脉反流和慢性肾脏疾病复杂化,但很少伴有肾病综合征。我们有一名患者患有大动脉炎并伴有主动脉瓣反流。她患有肾病综合征,免疫抑制治疗无效,但在主动脉瓣置换术后病情迅速好转。在她的肾活检中,肾小球有轻微的系膜增生性变化,没有免疫复合物沉积。她的蛋白尿一直呈阴性,直到瓣膜周围渗漏导致主动脉瓣反流复发。手术17年后,她突然去世了。在她的肾脏尸检中,小动脉显示严重的透明质增生,肾小球显示系膜增生性变化,伴有节段性血管松解。严重的主动脉瓣反流可能改变了肾脏血流动力学并引起肾小球病变,从而导致肾病综合征。我们应该注意高动脉炎合并主动脉瓣反流患者肾病综合征的罕见但关键的合并症。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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