We read with interest the article by Ng et al1 comparing 18 consecutive patients with senile systemic amyloidosis (SSA) with a randomly selected group of patients with light chain-associated (AL) amyloidosis. The authors emphasize that SSA is a disorder of elderly men and that the male predilection seems to be unique to SSA (whereas the male-female ratios in the mutant transthyretin [ATTR] and AL forms of amyloidosis are about 50:50 and 60:40, respectively). As the authors remark, “this intriguing observation raises the possibility of a factor in males that predisposes them to the deposition of wild-type transthyretin in the heart or, alternatively, suggests an inherent protective factor in females.”1(p1429) Data from our hereditary and acquired amyloidosis center confirm this observation and could extend the hypothesis of sex-related factors(s) that are able to modulate development of amyloidotic cardiomyopathy to patients with mutant, as well as wild-type, transthyretin.
Rapezzi C, Perugini E, Salvi F. Female Sex as a Possible Protective Factor From Myocardial Involvement in Transthyretin-Related Amyloidoses. Arch Intern Med. 2005;165(20):2429. doi:10.1001/archinte.165.20.2429