amyloidosis

• rheumatology

Systemic diseases with secondary glomerular involvement

• overview 󰒖

• lightchian disease

• Deposition of misfolded proteins as insoluble fibrils (β-pleated sheets) in normal tissues

• 最常見的四種系統性分別是 AL、AA、Ab2M、和遺傳與老年相關的（ATTR）

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• clinical manifestations of amyloidosis

• diagnostic studies of amyloidosis 󰒗

• primary amyloidosis (AL)

• treatment of amyloidosis

• Clearance of amyloid by Ab under study

  • (NEJM 2015;373:1106; Br J Haematol 2020;189:228)

• Cardiac:

  • diuretics;
  • avoid CCB;
  • ↑ dig toxicity risk (? amyloid binding);
  • anticoag all AF

• Heart, kidney, and liver transplant

  • may be considered in those w/ advanced disease

• Median survival:

  • 5y AL (~6 m if CM);
  • 11y AA;
  • 4y wt ATTR & 2.5y ATTR V122I CM w/o Rx