Schaeffer J, Floege J, Ehlerding G, Koch K M
Division of Nephrology, Hannover Medical School, Germany.
Nephrol Dial Transplant. 1995;10 Suppl 3:4-8. doi: 10.1093/ndt/10.supp3.4.
Dialysis-related amyloidosis (DRA) is a major cause of morbidity in end-stage renal disease patients. While retention of the precursor protein beta 2-microglobulin (beta 2-m) forms the essential basis for DRA, pathogenetic concepts include: qualitative and quantitative alterations in beta 2-m metabolism; local and systemic inflammatory changes, partly related to different treatment modes; general predisposing factors such as age at the onset of dialysis treatment. Clinical and radiological signs, as well as synovial thickening on sonography, suggest the presence of DRA, but histomorphological demonstration of beta 2m-amyloid is required for definitive proof. Scintigraphic imaging of DRA represents an additional, sensitive non-invasive diagnostic tool. Successful kidney transplantation stops the progression of DRA.
透析相关性淀粉样变性（DRA）是终末期肾病患者发病的主要原因。虽然前体蛋白β2-微球蛋白（β2-m）的潴留是DRA的基本发病基础，但发病机制概念包括：β2-m代谢的定性和定量改变；局部和全身炎症变化，部分与不同的治疗方式有关；一般易感因素，如开始透析治疗时的年龄。临床和放射学体征以及超声检查显示的滑膜增厚提示DRA的存在，但确诊需要β2m淀粉样变的组织形态学证据。DRA的闪烁显像代表了一种额外的、敏感的非侵入性诊断工具。成功的肾移植可阻止DRA的进展。
