Yamamoto Suguru, Gejyo Fumitake
Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Science, 757 Asahimachi-dori, Niigata 951-8510, Japan.
Biochim Biophys Acta. 2005 Nov 10;1753(1):4-10. doi: 10.1016/j.bbapap.2005.09.006. Epub 2005 Sep 30.
Dialysis-related amyloidosis (DRA) is a frequent and serious complication in patients on long-term dialysis. The amyloid has a marked affinity for joint tissues, and carpal tunnel syndrome, polyarthralgia, destructive spondyloarthropathy, and bone cysts are the major clinical manifestations of DRA. beta(2)-Microglobulin (beta(2)-m) was identified as the major protein constituent of the amyloid fibrils. Risk factors for the development of DRA include age, duration of dialysis treatment, use of low-flux dialysis membrane, use of low purity dialysate, monocyte chemoattractant protein-1 GG genotype, and apolipoprotein E4 allele, although the retention of beta(2)-m in the plasma appears to be prerequisite. Clinical therapeutic strategies for DRA include dialysis, medical or surgical therapy, and renal transplantation. Preventive measures have attempted to remove beta(2)-m from the serum by using high-flux membranes and a beta(2)-m adsorption column in hemodialysis. Renal transplantation is a radical approach to treating the arthralgias attributed to the amyloid deposits while the regression of dialysi-related amyloid deposits is not identified after successful renal transplantation in many studies. It is necessary to elucidate the pathogenesis of DRA and to establish more effective prevention and therapy in the future.
透析相关性淀粉样变性（DRA）是长期透析患者常见且严重的并发症。该淀粉样物质对关节组织具有显著亲和力，腕管综合征、多关节痛、破坏性脊椎关节病和骨囊肿是DRA的主要临床表现。β2微球蛋白（β2 -m）被确定为淀粉样纤维的主要蛋白质成分。DRA发生的危险因素包括年龄、透析治疗时长、使用低通量透析膜、使用低纯度透析液、单核细胞趋化蛋白-1 GG基因型和载脂蛋白E4等位基因，不过血浆中β2 -m的潴留似乎是前提条件。DRA的临床治疗策略包括透析、药物或手术治疗以及肾移植。预防措施试图通过在血液透析中使用高通量膜和β2 -m吸附柱从血清中清除β2 -m。肾移植是治疗由淀粉样沉积物引起的关节痛的根本方法，但在许多研究中，成功肾移植后并未发现透析相关淀粉样沉积物有所消退。未来有必要阐明DRA的发病机制并建立更有效的预防和治疗方法。
