Recent progress in understanding dialysis-related amyloidosis.

摘要

Dialysis-Related Amyloidosis (DRA) is a general amyloidosis, which is specifically found in CKD stage 5 patients. DRA causes various osteoarticular lesions in dialysis patients, and therefore it is not practical to regard this condition separately from chronic kidney disease-mineral and bone disorder (CKD-MBD), at least from the viewpoint of daily clinical practice. However, it is still controversial whether this disease condition should be included in CKD-MBD. Recently, a better understanding of the pathogenesis of DRA has been obtained by examination of beta(2)-microglobulin-related amyloid fibril formation, extension, and depolymerization in vitro. Apoliprotein E, proteoglycans, and glycosaminoglycans stabilize the amyloid fibrils. In addition, some lysophospholipids and non-esterified fatty acids accelerate amyloid fibril formation and extension under physiological conditions in vitro. Those molecules may enhance the amyloid deposition in vivo. The frequency and severity of osteoarticular disorders that may be associated with DRA accelerate with the duration of dialysis therapy. We have shown that patients undergoing dialysis therapy for 30 years or more survive with serious complications from osteoarticular disorders. DRA is one of the most harmful osteoarticular complications with regard to the maintenance of daily activities and quality of life in patients undergoing long-term dialysis therapy, in addition to the classical complications of CKD-MBD.