Thorne Jordan, Clark David, Geldenhuys Laurette, More Keigan, Vinson Amanda, Tennankore Karthik
Department of Medicine, Dalhousie University and Nova Scotia Health.
Division of Nephrology, Nova Scotia Health.
Kidney Med. 2022 Jun 26;4(8):100504. doi: 10.1016/j.xkme.2022.100504. eCollection 2022 Aug.
Serum amyloid A protein (AA) amyloidosis, also known as secondary amyloidosis, is a known consequence of chronic inflammation and results from several conditions including inflammatory arthritis, periodic fever syndromes, and chronic infection. AA amyloidosis can lead to multiorgan dysfunction, including changes in glomerular filtration rate and proteinuria. Definitive diagnosis requires tissue biopsy, and management of AA amyloid kidney disease is primarily focused on treating the underlying inflammatory condition to stabilize glomerular filtration rate, reduce proteinuria, and slow potential progression to kidney failure. In this narrative review, we describe the causes, pathophysiology, presentation, and pathologic diagnosis of AA amyloid kidney disease using an illustrative case of biopsy-proven AA amyloid kidney disease in a patient with long-standing rheumatoid arthritis who had a favorable response to interleukin 6 inhibition. We conclude the review with a description of established and more novel therapies for AA amyloidosis including published cases of use of tocilizumab (an interleukin 6 inhibitor) in biopsy-proven AA amyloid kidney disease.
血清淀粉样蛋白A（AA）淀粉样变性，也称为继发性淀粉样变性，是慢性炎症的已知后果，由多种疾病引起，包括炎性关节炎、周期性发热综合征和慢性感染。AA淀粉样变性可导致多器官功能障碍，包括肾小球滤过率改变和蛋白尿。明确诊断需要组织活检，AA淀粉样肾病的治疗主要集中在治疗潜在的炎症性疾病，以稳定肾小球滤过率、减少蛋白尿并减缓向肾衰竭发展的可能性。在这篇叙述性综述中，我们通过一个活检证实为AA淀粉样肾病的病例来说明其病因、病理生理学、临床表现和病理诊断，该患者患有长期类风湿关节炎，对白介素6抑制治疗反应良好。我们在综述结尾描述了已确立的和更新颖的AA淀粉样变性治疗方法，包括在活检证实的AA淀粉样肾病中使用托珠单抗（一种白介素6抑制剂）的已发表病例。
