eMedicine Specialties > Nephrology > The Kidney in Systemic Diseases
Amyloidosis, Beta2M (Dialysis-Related): Treatment & Medication
Updated: Feb 22, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
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.
- 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.
- 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.
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.
Consultations
- A rheumatology specialist may be needed.
- Initiate early consideration for renal transplantation.
Diet
- No known diet directly affects beta-2-microglobulin.
- Patients with renal failure should receive the appropriate dietary management for that condition.
Medication
No medical treatment presently exists to reverse or alter the disease course. Low-dose steroids and nonsteroidal anti-inflammatory agents are symptomatic approaches in ameliorating joint pain and inflammation.
Immunosuppressive agents
Used to suppress the inflammatory process.
Prednisone (Deltasone, Sterapred, Orasone)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Used only in severe cases of joint pain and immobility.
Adult
Up to 60 mg PO qd, taper as symptoms resolve
Pediatric
Not established
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; concomitant NSAID use can increase risk of GI bleed; decreases effects of aspirin and toxoids (for immunizations); results of skin testing to antigens (eg, tuberculosis) may be falsely negative
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in leukopenia or thrombocytopenia (can cause lowering of blood counts with a prolonged recovery phase); abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Triamcinolone (Aristospan Intra-articular)
Decreases inflammation by suppressing migration of PMNs and reducing capillary permeability. Decreases autoimmune reactions, possibly by suppressing key components of the immune system.
Adult
40 mg intra-articularly once; may repeat in 3 mo
Pediatric
Not established
Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
Documented hypersensitivity; viral, fungal, or tubercular skin lesions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Multiple complications (eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression) may occur; abrupt discontinuation of glucocorticoids may cause adrenal crisis
Topical analgesics
Penetrate deep for temporary relief of minor aches and pains of muscles and joints associated with arthritis.
Capsaicin (Capzasin-P)
Derived from plants of Solanaceae family. May render skin and joints insensitive to pain by depleting substance P in peripheral sensory neurons.
Adult
Apply to affected area tid/qid (wash hands with soap and water after applying)
Pediatric
Not established
None reported
Documented hypersensitivity; broken or irritated skin
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
For external use only; avoid contact with eyes; do not use a tight bandage; discontinue use if condition worsens or symptoms persist for 14-28 d; not for use with heating pad
Nonsteroidal anti-inflammatory agents
These agents have analgesic, anti-inflammatory properties and antipyretic activities. Their mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.
Ibuprofen (Advil, Excedrin IB, Ibuprin, Motrin)
DOC for patients with mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
Adult
400 mg PO q4-6h, 600 mg q6h, or 800 mg q8h while symptoms persist; not to exceed 3.2 g/d
Pediatric
Not established
Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; simultaneous administration with low-dose aspirin may decrease aspirin's cardioprotective and stroke preventive effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, beta-blockers, and diuretic effect of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin or lithium serum levels
Documented hypersensitivity; peptic ulcer disease, recent GI bleeding or perforation, renal insufficiency, or high risk of bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Category D in third trimester of pregnancy; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy
Sulindac (Clinoril)
Decreases activity of cyclooxygenase and, in turn, inhibits prostaglandin synthesis. Results in decreased formation of inflammatory mediators.
Adult
150-200 mg PO bid prn
Pediatric
Not established
Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Documented hypersensitivity; sensitivity to aspirin, iodides, or other NSAIDs; GI bleed; renal insufficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in preexisting renal disease or compromised renal perfusion; low white blood cell counts occur rarely and usually return to the reference range in ongoing therapy; discontinuation of therapy may be necessary in persistent leukopenia, granulocytopenia, or thrombocytopenia; caution in patients with anticoagulation defects or patients who are receiving anticoagulant therapy
More on Amyloidosis, Beta2M (Dialysis-Related) |
| Overview: Amyloidosis, Beta2M (Dialysis-Related) |
| Differential Diagnoses & Workup: Amyloidosis, Beta2M (Dialysis-Related) |
 Treatment & Medication: Amyloidosis, Beta2M (Dialysis-Related) |
| Follow-up: Amyloidosis, Beta2M (Dialysis-Related) |
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Further Reading
Keywords
dialysis-related amyloidosis, DRA, hemodialysis-associated amyloidosis, beta2 -microglobulin amyloidosis, beta-2-microglobulin amyloidosis, hemodialysis, HD, continuous ambulatory peritoneal dialysis, CAPD, near end-stage renal disease, carpal tunnel syndrome, flexor tenosynovitis, trigger finger, trigger thumb, scapulohumeral arthropathy, spondyloarthropathy, bone cysts, pathologic fractures, bone cysts
