List 5 features of amyloidosis in an HD patient. (5 points)
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Answer from UpToDate

• accumulation and tissue deposition of amyloid fibrils consisting of beta2-microglobulin (beta2-m) in the bone, periarticular structures, and viscera of patients with chronic kidney disease (CKD
• prevalence of DRA has decreased with the use of high-flux biocompatible membranes, which provide better clearance of beta2-m and are less likely to induce reactive inflammation
• Amyloid deposition results in the following common presentations:

●Carpal tunnel syndrome (CTS)

●Scapulohumeral periarthritis

●Flexor tenosynovitis

●Destructive spondyloarthropathy

●Bone cysts

●Visceral involvement, particularly gastrointestinal

• lesions in which beta2-m amyloid is deposited are associated with a marked influx of activated macrophages expressing cytokines, such as interleukin-1, tumor necrosis factor-alpha, and transforming growth factor-beta
• Beta2-m may cause bone destruction by directly stimulating formation of osteoclasts

• A prospective postmortem study found joint amyloid deposition in 21 percent of patients receiving hemodialysis for <2 years, 50 percent at 4 to 7 years, 90 percent at 7 to 13 years, and 100 percent at >13 years
• Risk factors for DRA include the following
○ ●Increasing age and dialysis vintage
○ ●Use of low-flux dialysis membranes
○ ●Use of bioincompatible dialysis membrane
○ ●Lack of residual renal function

• Physical exam - Guitar string hand - fixed flexion deformity of the hand. Amyloid hand - decreased prominence of thenar eminence.
• renal transplantation since it provides the most effective reduction of beta2-m levels

• Rx
○ hemodialysis patients with DRA, we use a highly biocompatible, high-flux membrane
○ increase the dialysis duration and/or frequency. Increasing the weekly treatment time decreases beta2-m concentrations
○ consider switching to either nocturnal or short daily hemodialysis since both modalities may be better than conventional thrice weekly dialysis for reducing beta2-m

○ Significant removal of beta2-m occurs with hemofiltration and hemodiafiltration

○ use of a beta2-m column that is available for clinical use in Japan in patients with DRA is associated with increased beta2-m removal and better improvement in symptoms versus dialysis alone
○ PD - May or may not have lower incidence - confounded by RRF

○ Surgery and analgesia helpful - in surgical treatments removal of the beta2 m infiltrated synovium is advisable.
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