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Dialysis-Associated Amyloidosis

  • Paweena Susantitaphong
  • Laura M. Dember
  • Bertrand L. JaberEmail author
Part of the Current Clinical Pathology book series (CCPATH)

Abstract

Dialysis-associated amyloidosis resulting from the accumulation of beta-2 microglobulin (β2m) is an important long-term complication in patients with chronic kidney failure on long-term dialysis. Bone and peri-articular tissue are the most common sites of β2m deposition. Typical clinical manifestations include the carpal tunnel syndrome, chronic arthropathy, spondyloarthropathies, subchondral bone cysts, and fractures. Although the pathogenesis of dialysis-associated amyloidosis has not been fully elucidated, there are several relevant molecular pathways that have been identified. Histopathological examination is the gold standard for diagnosing β2m-related amyloidosis. The diagnosis is made on the basis of positive Congo red staining with typical green-yellow birefringence under polarized light, coupled with positive anti-β2m antibody immuno-staining or amyloid protein identification by proteomic methods. The β2m amyloid deposition starts early in the course of the disease, long before the development of symptoms. Therefore, early detection especially in high-risk patients may be important to prevent or delay disease progression before the development of debilitating complications. Novel dialysis modalities to enhance β2m removal and ultrapure dialysate to reduce β2m synthesis from chronic inflammation are treatment strategies for dialysis-related amyloidosis. However, successful kidney transplantation remains the only potential curative treatment of choice.

Keywords

Dialysis Hemodialysis amyloidosis Beta-2 microglobulin Arthropathy Bone cysts 

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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Paweena Susantitaphong
    • 1
    • 2
  • Laura M. Dember
    • 3
  • Bertrand L. Jaber
    • 1
    Email author
  1. 1.Division of Nephrology, Department of MedicineSt. Elizabeth’s Medical Center, Tufts University School of MedicineBostonUSA
  2. 2.Extracorporeal Multiorgan Support Dialysis Center, Division of Nephrology, Department of MedicineKing Chulalongkorn Memorial Hospital, Chulalongkorn UniversityBangkokThailand
  3. 3.Renal-Electrolyte and Hypertension DivisionPerelman School of Medicine, University of PennsylvaniaPhiladelphiaUSA

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