β₂-microglobulin amyloidosis

Abstract

β₂-microglobulin (β2M) amyloidosis is a disabling disease that affects patients with long-term chronic renal failure but not individuals with normal renal function. It is an ideal model for study of the amyloid diseases because a defined population is at risk for the development of this condition, its clinical manifestations evolve predictably over time, and it ultimately occurs in the majority of patients who have received long-term dialysis therapy. The clinical features of β2M amyloidosis are reviewed, highlighting the systemic nature of this disease. The various techniques available to diagnose this condition are described. The pathogenesis of β2M amyloidosis is reconsidered in light of recently published data regarding advanced glycation end products (AGE). Available and potential treatments for this condition are also discussed.

Key Words:

• β₂-microglobulin amyloidosis
• dialysis-related amyloidosis
• hemodialysis
• uremia
• advanced glycosylation end products
• carpal tunnel syndrome
• shoulder periarthritis
• bone cysts
• destructive spondyloarthropathy
• renal transplantation
• aminoguanidine