Division of Nephrology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Immunology, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey.
Curr Rheumatol Rep. 2024 Jul;26(7):248-259. doi: 10.1007/s11926-024-01147-8. Epub 2024 Apr 3.
Amyloid A (AA) amyloidosis is an organ- or life-threatening complication of chronic inflammatory disorders. Here, we review the epidemiology, causes, pathogenesis, clinical features, and diagnostic and therapeutic strategies of AA amyloidosis.
The incidence of AA amyloidosis has declined due to better treatment of the underlying diseases. Histopathological examination is the gold standard of diagnosis, but magnetic resonance imaging can be used to detect cardiac involvement. There is yet no treatment option for the clearance of amyloid fibril deposits; therefore, the management strategy primarily aims to reduce serum amyloid A protein. Anti-inflammatory biologic agents have drastically expanded our therapeutic armamentarium. Kidney transplantation is preferred in patients with kidney failure, and the recurrence of amyloidosis in the allograft has become rare as transplant recipients have started to benefit from the new agents. The management of AA amyloidosis has been considerably changed over the recent years due to the novel therapeutic options aiming to control inflammatory activity. New agents capable of clearing amyloid deposits from the tissues are still needed.
淀粉样 A(AA)淀粉样变性是慢性炎症性疾病的一种危及器官或生命的并发症。本文综述了 AA 淀粉样变性的流行病学、病因、发病机制、临床特征以及诊断和治疗策略。
由于基础疾病治疗的改善,AA 淀粉样变性的发病率有所下降。组织病理学检查是诊断的金标准,但磁共振成像可用于检测心脏受累。目前尚无清除淀粉样纤维沉积的治疗选择;因此,管理策略主要旨在减少血清淀粉样 A 蛋白。抗炎症生物制剂极大地扩展了我们的治疗手段。对于肾衰竭患者,肾移植是首选,由于移植受者开始受益于新的治疗药物,移植物中淀粉样变性的复发已变得罕见。由于新型治疗药物旨在控制炎症活性,AA 淀粉样变性的治疗在近几年发生了重大变化。仍需要能够清除组织中淀粉样沉积物的新型药物。