Department of Rheumatology & Clinical Immunology, AA21, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands.
Rheum Dis Clin North Am. 2013 May;39(2):323-45. doi: 10.1016/j.rdc.2013.02.012. Epub 2013 Mar 13.
Amyloidosis is the name for protein-folding diseases characterized by extracellular deposition of a specific soluble precursor protein that aggregates in the form of insoluble fibrils. The classification of amyloidosis is based on the chemical characterization of the precursor protein. Deposition of amyloid is localized or systemic. The 4 main types of systemic amyloidosis are AL, AA, ATTR, and Aβ2M type. A schematic approach is proposed for the clinical management of systemic amyloidosis. The importance of typing amyloid with confidence, the usefulness of imaging techniques, the principles of treatment, and the need for well-planned treatment monitoring during follow-up are discussed.
淀粉样变性是一种由特定可溶性前体蛋白细胞外沉积引起的蛋白质折叠疾病,这些蛋白以不溶性纤维的形式聚集。淀粉样变性的分类基于前体蛋白的化学特征。淀粉样物质的沉积是局部的或全身性的。4 种主要的系统性淀粉样变性分别为 AL、AA、ATTR 和 Aβ2M 型。本文提出了一种系统性淀粉样变性的临床管理的示意图方法。讨论了明确分型的重要性、影像学技术的作用、治疗原则以及在随访期间需要进行精心计划的治疗监测。