Amyloidosis type | Clinical presentation | Clinical outcome |
---|---|---|
AL | Heart failure, often with multiorgan involvement especially hepatomegaly, peripheral and autonomic neuropathy, nephrotic syndrome, macroglossia and periorbital purpura | With treatment and haematological response, patients’ outcomes vary with organ involvement; patients with advanced cardiac involvement may experience sudden cardiac death despite haematological response Without treatment, patients with cardiac involvement live <1 year |
ATTRm | Heart failure with peripheral or autonomic neuropathy; only 50% positive family history | Cardiomyopathy is slowly progressive, and patients may not present with symptoms until ventricular wall thickening causes functional deficits in cardiac function Without treatment, patients with ATTR cardiac amyloidosis can survive from years to decades |
ATTRwt | Heart failure with carpal tunnel syndrome, biceps tendon rupture and spinal stenosis Atrial fibrillation is a common feature, often several years before heart failure |
AL, amyloid light chain; ATTRm, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; LV, left ventricular.