Jurczyszyn Artur, Engel Anna, Rajzer Marek, Czepiel Jacek, Mazurs Grzegorz
Przegl Lek. 2014;71(6):340-5.
The heart is an organ often occupied by various forms of amyloidosis; cardiomyopathies are the leading cause of mortality in patients with amyloidosis. Cardiac amyloidosis is often diagnosed late because of nonspecific symptoms and missed early signs in the imaging routine. A method for treating cardiac amyloidosis depends on the type of amyloid protein. In the treatment of amyloidosis associated with immunoglobulins systemic chemotherapy is used without transplant or stem cell transplantation and in the treatment of familial transthyretin amyloidosis liver transplantation is used. There are still clinical studies on the use of siRNA for the treatment of cardiomyopathy associated with transthy retin amyloidosis, and on the use of amyloid protein stabilizers. The prognosis depends on the type of amyloid protein; worse results observed in the case of light chain amyloidosis. Care support is the cornerstone of treatment; it is expected that advances in cardiac imaging and proteomics positive impact on our ability to diagnosis, prognosis and treatment outcomes of amyloidosis of the heart.
心脏是一个常被各种形式淀粉样变性占据的器官；心肌病是淀粉样变性患者的主要死亡原因。由于非特异性症状以及影像学检查常规中早期体征的遗漏，心脏淀粉样变性常常在晚期才被诊断出来。治疗心脏淀粉样变性的方法取决于淀粉样蛋白的类型。在与免疫球蛋白相关的淀粉样变性治疗中，使用全身化疗而不进行移植或干细胞移植，而在家族性转甲状腺素蛋白淀粉样变性的治疗中，则使用肝移植。目前仍有关于使用小干扰RNA治疗与转甲状腺素蛋白淀粉样变性相关心肌病以及使用淀粉样蛋白稳定剂的临床研究。预后取决于淀粉样蛋白的类型；在轻链淀粉样变性的情况下观察到的结果更差。护理支持是治疗的基石；预计心脏成像和蛋白质组学的进展将对我们诊断、预测和治疗心脏淀粉样变性的能力产生积极影响。
