Cardiac AA amyloidosis in a patient with rheumatoid arthritis and systemic sclerosis: the therapeutic potential of biological reagents.

摘要

Reactive amyloid A (AA) amyloidosis is a serious and life-threatening systemic complication of rheumatoid arthritis (RA) (1), and the AA amyloid deposition usually affects the kidneys and gastrointestinal tract (2-4). Unlike AL amyloidosis, the prevalence of cardiac involvement in reactive AA amyloidosis is low, affecting only about 10% of patients, and clinically overt heart failure is usually present in the terminal phase of the disea-se course in addition to end-stage renal disease (ESRD) (5). In general, the prognosis of patients with cardiac amyloidosis is known to be poor, with an average survival time of 1 to 2 years after diagnosis, and can even be as short as 6 months once clinically overt heart failure has appeared (6).