Systemic amyloidosis: a review with emphasis on pathogenesis

Our knowledge about the composition of the deposits in amyloidosis has increased considerably during the last decade. Three different protein groups have been shown to form fibrils in systemic amyloidosis, namely monoclonal immunoglobulin light chains in primary and myeloma-associated amyloidosis, protein AA in secondary amyloidosis and prealbumin in the familial and senile forms of systemic amyloidosis. This review deals with known and postulated pathogenetic mechanisms involved in the creation of fibrils from these proteins.