LA2019-09-22T17:57:21+00:00

Localised Amyloidosis

The section Localised Amyloidosis is under construction and will be completed by the end of the year.
A summary is presented below.

Introduction

  • Localized amyloidosis is a disorder characterized by single non-vital organ or tissue specific production and deposition of amyloid
  • The amyloid consists of a variety of constitutive proteins but most commonly immunoglobulin light chain
  • Localised amyloid usually involves the mucosal, cutaneous or glandular tissues
  • In a study of 65 AAN clinic patients with localized amyloidosis the sites involved were
    • 29% respiratory (mainly larynx)
    • 19% genitourinary (mainly bladder)
    • 16% GIT (mainly stomach and duodenum)
    • 12% ophthalmic (mainly conjunctiva)
    • 8% skin
    • 3% auditory
  • Symptoms are generally related to local mass effect or bleeding, with excellent response to local surgical intervention alone
  • Thorough testing should be conducted to exclude systemic amyloidosis by organ staging, monoclonal gammopathy screening and other tests to exclude systemic AL, ATTRwt, inherited and AA amyloidosis
  • Localised amyloidosis has significantly better prognosis than systemic AL amyloidosis and does not require systemic chemotherapy with local surgical intervention providing good disease control