The two major kinds of amyloidosis are:
1) Primary amyloidosis (AL) (Amyloid Light chain)
2) Secondary amyloidosis (AA)
AA fibrils are derived by proteolysis from a larger precursor in the serum called SAA (serum amyloid–associated) protein that is synthesized in the liver under the influence of cytokines such as IL-6 and IL-1. The production of SAA protein is increased in inflammatory states as part of the “acute phase response”; therefore, this form of amyloidosis is associated with chronic inflammation. However, increased production of SAA by itself is not sufficient for the deposition of amyloid. You need to have an enzyme defect that results in incomplete breakdown of SAA, thus generating insoluble AA molecules.
Treatment: Treat the underlying source of inflammation. Eprodisate is a member of a new class of compounds that inhibits polymerization of amyloid fibrils potentially slowing the decline in renal function
Posted by Tarek Alhamad