Amyloidosis, Beta2M (Dialysis-Related) Treatment & Management

  • Author: Anita Basu, MD; Chief Editor: Vecihi Batuman, MD, FACP, FASN   more...
 
Updated: Jun 3, 2010
 

Medical Care

At present, no adequate treatment of beta-2-microglobulin amyloidosis exists. Medical therapy is limited to symptomatic approaches in ameliorating joint pain and inflammation.

  • The treatment of joint pain includes nonsteroidal anti-inflammatory drugs, intra-articular injections of prednisolone, 10% hydrocortisone cream, and low-dose oral prednisone in severe cases.
  • Conservative treatment includes physical and occupational therapy. Wrist splints, cervical collars, lumbar corsets, knee braces, and immobilization for spondyloarthropathies often are helpful.
  • Improvement of dialysis membranes: High-flux biocompatible polyacrylonitrile and polysulfone membranes have increased middle molecule removal and thereby enhance beta-2-microglobulin removal during HD and hemofiltration.
  • Online hemodiafiltration: This has been associated with the maximal removal of beta-2-microglobulin.[13]
  • Dialysate preparations: Use of ultrapure, sterile, and apyrogenic dialysate may aid in decreasing stimulation and in releasing cytokines. It also may decrease plasma levels of acute phase proteins.[14]
  • Direct hemoperfusion-type adsorption column (Lixelle): This was developed to selectively eliminate beta-2-microglobulin from the circulating blood of patients with DRA. Lixelle treatments reduce the circulating levels of beta-2-microglobulin and inflammatory cytokines, thereby improving the symptoms of patients with DRA. While these treatments have been used and studied in Japan, they are not currently used in the United States.[15, 16]
  • Studies in Japan have been done to destroy amyloid fibrils of a beta2-microglobulin fragment by laser beam irradiation; this has implications in both preventing the deposition of amyloid fibrils and for destroying preformed amyloid deposits.[17]
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Surgical Care

Surgical intervention, including carpal tunnel release with surgical decompression of the median nerve or release of the transverse carpal ligaments under endoscopic visualization, flexor tenosynovectomy or percutaneous first annular pulley release, spinal stabilization or laminectomy, or total joint replacement, may be effective in alleviating pain and restoring function. Unfortunately, orthopedic interventions have high failure rates in DRA compared with the general population. If during the course of a surgery, beta-2-microglobulin amyloidosis is suspected, then a biopsy should be performed at that time.

  • Renal transplantation
    • Renal transplantation is the treatment of choice for beta-2-microglobulin amyloidosis. It lowers the blood concentration of beta-2-microglobulin to the reference range, halting the progression of the disease.
    • Osteoarticular symptoms, such as joint pain, swelling, and stiffness, disappear within the first week after transplantation. Cystic lesions usually remain unchanged, and regression of amyloid deposits probably does not occur.
    • Transplantation is not an option for all patients. Renal transplantation has already failed in some patients on long-term dialysis when they develop beta-2-microglobulin amyloidosis, and others may not be suitable candidates.
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Consultations

  • A rheumatology specialist may be needed.
  • Initiate early consideration for renal transplantation.
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Diet

  • No known diet directly affects beta-2-microglobulin.
  • Patients with renal failure should receive the appropriate dietary management for that condition.
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Contributor Information and Disclosures
Author

Anita Basu, MD  Assistant Professor of Medicine, University of Mississippi School of Medicine; Staff Nephrologist, GV (Sonny) Montgomery Veterans Affairs Medical Center

Anita Basu, MD is a member of the following medical societies: American College of Physicians and National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Carol A Bogdan, MD  Consulting Staff, Coastal Cancer Center, Myrtle Beach, SC

Disclosure: Nothing to disclose.

Reynaldo Matute, MD  Clinical Assistant Professor, Department of Internal Medicine, Division of Nephrology, New York Medical College

Reynaldo Matute, MD is a member of the following medical societies: American Society of Nephrology and National Kidney Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Donald A Feinfeld, MD, FACP, FASN  Consulting Staff, Division of Nephrology & Hypertension, Beth Israel Medical Center

Donald A Feinfeld, MD, FACP, FASN is a member of the following medical societies: American Academy of Clinical Toxicology, American Society of Hypertension, American Society of Nephrology, and National Kidney Foundation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

George R Aronoff, MD  Director, Professor, Departments of Internal Medicine and Pharmacology, Section of Nephrology, Kidney Disease Program, University of Louisville School of Medicine

George R Aronoff, MD is a member of the following medical societies: American Federation for Medical Research, American Society of Nephrology, Kentucky Medical Association, and National Kidney Foundation

Disclosure: Nothing to disclose.

Rebecca J Schmidt, DO, FACP, FASN  Professor of Medicine, Section Chief, Department of Medicine, Section of Nephrology, West Virginia University School of Medicine

Rebecca J Schmidt, DO, FACP, FASN is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Nephrology, International Society of Nephrology, National Kidney Foundation, Renal Physicians Association, and West Virginia State Medical Association

Disclosure: Abbott Grant/research funds Speaking and teaching; AMAG Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching; Ortho Biotech Honoraria Speaking and teaching; Renal Ventures Ownership interest Other

Chief Editor

Vecihi Batuman, MD, FACP, FASN  Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Medicine Service, Southeast Louisiana Veterans Health Care System

Vecihi Batuman, MD, FACP, FASN is a member of the following medical societies: American College of Physicians, American Society of Hypertension, American Society of Nephrology, and International Society of Nephrology

Disclosure: Nothing to disclose.

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