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Dialysis-Related Beta-2m Amyloidosis Treatment & Management

  • Author: Anita Basu, MD, FACP; Chief Editor: Vecihi Batuman, MD, FACP, FASN  more...
 
Updated: May 01, 2014
 

Approach Considerations

At present, no adequate treatment of beta-2m amyloidosis exists. Medical therapy is limited to symptomatic approaches to ameliorating joint pain and inflammation. Conservative treatment includes physical and occupational therapy. Wrist splints, cervical collars, lumbar corsets, knee braces, and immobilization for spondyloarthropathies often are helpful.

The treatment of joint pain includes the use of nonsteroidal anti-inflammatory drugs (NSAIDs), intra-articular injections of prednisolone, 10% hydrocortisone cream, and, in severe cases, low-dose oral prednisone.

Surgery

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 rates in the general population. If, during the course of a surgery, beta-2m amyloidosis is suspected, then a biopsy should be performed at that time.

Consultations

Involve rheumatologic, surgical, and transplant consultants early.

Follow-up

A nephrologist should care for patients with beta-2m amyloidosis on an ongoing basis.

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Renal Transplantation

Renal transplantation is the treatment of choice for beta-2m amyloidosis. It lowers the blood concentration of beta-2m 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. Some patients on long-term dialysis will have undergone unsuccessful renal transplantation before they first developed beta-2m amyloidosis; in certain other cases, patients are not suitable candidates.

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Prevention

Although preventive measures are hard to assess, possible ways of preventing, or at least decreasing, the incidence of DRA are the use of a high-flux dialyzer, online hemodiafiltration,[14] ultrapure dialysate,[15] and adsorbent columns.[16]

Based on guidelines from the Disease Outcomes Quality Initiative (DOQI), there are no indications for the routine monitoring of beta-2m.[17]

High-flux dialyzers

High-flux biocompatible polyacrylonitrile and polysulfone membranes have increased middle molecule removal and have thereby enhanced beta-2m removal during HD and hemofiltration.

Online hemodiafiltration

Online hemodiafiltration has been associated with maximal removal of beta-2m.[14]

Ultrapure dialysate preparations

The 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.[15]

Direct hemoperfusion-type adsorption column (Lixelle)

This was developed to selectively eliminate beta-2m from the circulating blood of patients with DRA. Lixelle treatments reduce the circulating levels of beta-2m and inflammatory cytokines, thereby improving the symptoms of patients with DRA. While this therapy has been used and studied in Japan, it is not currently employed in the United States.[16, 18]

Laser therapy

Studies examining the use of laser-beam irradiation to destroy amyloid fibrils of beta-2m fragments have been performed in Japan. This technique has implications for the prevention of amyloid fibril deposition and for the destruction of preformed amyloid deposits.[19]

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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

Carol A Bogdan, MD Consultant in Hematology-Oncology, 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, National Kidney Foundation

Disclosure: Nothing to disclose.

Chief Editor

Vecihi Batuman, MD, FACP, FASN Huberwald Professor of Medicine, Section of Nephrology-Hypertension, Tulane University School of Medicine; Chief, Renal Section, 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, International Society of Nephrology

Disclosure: Nothing to disclose.

Acknowledgements

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.

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 Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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