Seneschall Charlotte, Law Steven, Roufosse Candice, Woodham Sarah, Kousios Andreas
West London Renal and Transplant Centre, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
Centre for Inflammatory Disease, Department of Immunology and Inflammation, Faculty of Medicine, Imperial College, London, UK.
J Nephrol. 2024 May;37(4):1147-1152. doi: 10.1007/s40620-023-01845-z. Epub 2024 Jan 11.
Systemic amyloid A amyloidosis is a progressive condition in which sustained elevation of serum amyloid A protein concentration leads to widespread amyloid deposition resulting in multiorgan failure without treatment. The kidney is the most commonly affected organ, and renal amyloid A amyloidosis can cause nephrotic syndrome and chronic kidney disease (CKD) leading to end stage kidney disease (ESKD). Serum Amyloid A protein is produced in the liver in response to chronic inflammation, specifically by inflammatory cytokines, especially IL-6. Tocilizumab, a monoclonal antibody that targets the interleukin-6 receptor, has increasingly been of interest in treating amyloid A amyloidosis. We present a case of a 79-year-old male with long-term seronegative polyarthritis who was referred with gradual decline in kidney function and nephrotic range proteinuria. His renal biopsy showed amyloid A amyloidosis with significant interstitial fibrosis and tubular atrophy. He was commenced on monthly tocilizumab infusions and peritoneal dialysis with good clinical response and rapid resolution of his nephrotic state. This case adds to the current literature on the benefits of tocilizumab in treating amyloid A amyloidosis in patients with advanced CKD.
系统性淀粉样蛋白A淀粉样变性是一种进行性疾病,其中血清淀粉样蛋白A蛋白浓度持续升高会导致广泛的淀粉样蛋白沉积,如不治疗会导致多器官衰竭。肾脏是最常受影响的器官,肾淀粉样蛋白A淀粉样变性可导致肾病综合征和慢性肾脏病(CKD),进而发展为终末期肾病(ESKD)。血清淀粉样蛋白A蛋白是肝脏在慢性炎症反应中产生的,特别是由炎性细胞因子,尤其是白细胞介素-6产生。托珠单抗是一种靶向白细胞介素-6受体的单克隆抗体,越来越受到治疗淀粉样蛋白A淀粉样变性的关注。我们报告一例79岁男性,患有长期血清阴性多关节炎,因肾功能逐渐下降和肾病范围蛋白尿前来就诊。他的肾活检显示为淀粉样蛋白A淀粉样变性,伴有明显的间质纤维化和肾小管萎缩。他开始每月接受托珠单抗输注和腹膜透析,临床反应良好,肾病状态迅速缓解。该病例为目前关于托珠单抗治疗晚期CKD患者淀粉样蛋白A淀粉样变性益处的文献增添了内容。