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 the following:
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Nonsteroidal anti-inflammatory drugs (NSAIDs)
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Intra-articular injections of prednisolone
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10% hydrocortisone cream
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Low-dose oral prednisone in severe cases
Successful reduction of reduction of osteoarticular pain due to dialysis-related beta-2m amyloidosis has been reported with low-dose doxycycline for up to 1 year. [20]
Surgery
Surgical intervention may be effective in alleviating pain and restoring function. Procedures include the following:
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Carpal tunnel release with surgical decompression of the median nerve or release of the transverse carpal ligaments under endoscopic visualization
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Flexor tenosynovectomy or percutaneous first annular pulley release
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Spinal stabilization or laminectomy
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Total joint replacement
Unfortunately, orthopedic interventions have high failure rates in dialysis-related amyloidosis (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.
Apheresis device
In 2015, the US Food and Drug Administration (FDA) authorized the use of the first device to treat patients with DRA, the Lixelle Beta 2-microglobulin Apheresis Column (Kaneka Corp). During hemodialysis, the patient's blood passes through the device before entering the dialysis filter, and porous cellulose beads in the device bind to and remove beta 2-microglobin. The FDA granted the device a Humanitarian Use Device designation, which is given if the device diagnoses or treats a disease or condition that affects or is found in fewer than 4000 individuals in the United States each year. [21]
A study of 1314 Japanese patients undergoing dialysis for more than 10 years reported higher quality of life scores for patients with DRA receiving continuous treatment with Lixelle compared with patients with DRA complications who were not receiving Lixelle. [22]
Consultations and follow-up
Involve rheumatologic, surgical, and transplant consultants early. A nephrologist should care for patients with beta-2m amyloidosis on an ongoing basis.
Kidney Transplantation
Kidney 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 kidney transplantation before they first developed beta-2m amyloidosis; in certain other cases, patients are not suitable candidates.
Prevention
Although preventive measures are hard to assess, possible ways of preventing, or at least decreasing, the incidence of DRA are the use of the following:
Guidelines from the National Kidney Foundation's Kidney Disease Outcomes Quality Initiative (KDOQI) recommend against screening for beta2-microglobulin amyloidosis, including measurement of serum levels of beta2-microglobulin. [26]
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. [23]
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. [24]
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. [25, 27]
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. [28]
