Senile systemic amyloidosis: are regional differences real or do they reflect different diagnostic suspicion and use of techniques?

Amyloid deposits in the heart derived from transthyretin are commonly seen at autopsy in elderly patients. Massive deposition, causing senile systemic amyloidosis (SSA), is considered to be relatively uncommon and reported series suggest that SSA is considerably less common than amyloid light-chain (AL) amyloidosis. This article reviews the autopsy prevalence of transthyretin-derived cardiac amyloid, addresses whether there is definitive evidence for regional or ethnic differences and argues that the reported prevalence of SSA significantly underrepresents the true prevalence of the disease which may be as common, or commoner, than AL amyloidosis.