Vous êtes sur la page 1sur 9

Dialysis Amyloidosis

Hemanth P
Dialysis Tutor
B N Patel Institute of Paramedical & Science
• Build up of amyloid proteins in the organs of human body.
• Systemic Amyloidosis:
– A Amyloidosis (AA)
– Light Chain Amyloidosis (AL)
– Heavy Chain Amylodosis (AH)
– Transthyretin Amylodosis
– Beta2-microglobulin amyloidosis (Abeta2M)
– Cryopyrin associalted periodic syndrome (CAPS) associated
Amyloidosis.
• Renal Involvement of Amyloidosis occurs mainly in AA and AL
amyloidosis.
• Deposition of ABeta2M occurs in patients on prolonged
dialysis.
• Diagnosis:
– Family history
– Evidence of renal dysfunction.
– Microscopic evaluation.
Dialysis Associated Amyloidosis

• Beta-2-Microglobulin amylodosis is a disabling condition that


affects patients undergoing long-term hemodialysis and
peritoneal dialysis.
• Evolves predictably overtime and is rare in the first few years
of HD.
• Invades synovial membranes and osteoarticular sites.
• Serum Beta2M – 1.5-3 mg/L.
Membranes

• Cellulose acetate and cuprophan membranes cannot clear


molecular wt. higher than 200 D
• Newer cellulose triacetate dialyzers and the high-flux
synthetic dialyzers do a better removal of beta2-M.
• Acetate and/or endotoxin may enter the blood via the dialyzer
and stimulate the release of cytokines, inducing beta2-M
production.
Clinical Manifestation

• Beta2-M almost never appear before a patient has undergone


5 years of dialysis.
• Patients often present with a characteristics triad of
– Carpal tunnel syndrome
– Shoulder pain
– Flexor tenosynovitis in hands.
Diagnosis

• Clinical appearance on tissues or bone biopsy.


• Synovium biopsy followed by routine hematoxylin and eosin
staining reveals homogeneous eosinophilic material.
• Amyloid deposits are positive for Congo red staining , showing
green briefringence of the amyloid fibrils under polarized
light.
• Immunostaining with Monoclonal anti-beta-2m antibody
confirms the diagnosis of beta-2M amyloidosis.
• Antisera to amyloid beta-2m are taken up by the Congo red-
positive areas, but are not taken up in other types of
amyloidosis.
Treatment

• Renal transplantation is the treatment of choice for beta-2m


amyloidosis.
• Osteoarticular symptoms disappears within the first week
after transplantation.
• Doxycycline:
– Inhibits beta-2m and inhibits accumulation in bone architecture.
– Low dose of 100mg of doxycycline daily helps in pain reduction,
increased range of motion, and no adverse effects in long term dialysis
patients with severe amyloid arthropathy.
• Beta-2m adsorbing columns for dialysis.
• No treatment presently exists to reverse or alter the course of
beta-2m amyloidosis. Low dose steroids and nonsteroidal
anti-inflammatory drugs (NSAIDs) are symptomatic
approaches to ameliorating joint pain and inflammation.
– Prednisone
– Triamcinolone
– Capsaicin topical
– Ibuprofen
– sulindac