Nephrol Dial Transplant (2000) 15: 17-24
© 2000 European Renal Association-European Dialysis and Transplant Association
Toxic Proteins
ß2-Microglobulin and amyloidosis
INSERM Unit 90 and Department of Nephrology, Hôpital Necker, Paris, France
Abstract
Dialysis-associated amyloidosis is a serious complication in chronic dialysis patients. Its clinical expression in terms of arthralgias, destructive arthopathies and carpal tunnel syndrome is often associated with amyloid deposits, which are mainly composed of ß2-microglobulin (ß2-M) fibrils, but in addition contain a number of other compounds. It is probable that ß2-M-amyloid deposition is related, at least in part, to the elevated plasma ß2-M that is characteristic of chronic renal failure. The latter can decrease with high-performance dialysis techniques but cannot be reduced to the normal range. Almost certainly, several other systemic and local factors are involved, including ß2-M transformed by advanced glycation end products and advanced oxidation protein products, serum P component, ubiquitin, calcium crystals, cytokines, immunoglobulin light chains, proteases and antiproteases, as well as modified collagen and glucosaminoglycans. It is also possible that the ß2-M protein, in its native or modified form, exerts noxious effects on bone and joint tissues, in addition to its mere ‘passive’ presence as amyloid fibrils. Several retrospective studies and one prospective study suggest that dialysis strategies with highly permeable, synthetic membranes and/or ultrapure dialysate may be partially protective or at least delay the onset of dialysis amyloidosis. Successful kidney transplantation generally halts the disease process and leads to rapid relief of osteoarticular pain although regression of ß2-M-amyloid deposits probably does not occur.
Notes
Correspondence and offprint requests to: T. B. Drtüeke, MD, INSERM Unité 90 et Département de Néphrologie, Hôpital Necker, 161 rue de Sèvres, 75743 Paris Cedex 15, France.
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