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