Primary amyloidosis as a cause of microvascular angina and intermittent claudication.
Primary systemic amyloidosis or AL amyloidosis is a rare condition characterized by extracellular deposits of fibrils composed of fragments of immunoglobulin light chains. Widespread deposition of this amyloid in tissues interferes with their normal function and leads to multiple organ failure. Clinical manifestations are highly variable due to the wide range of organs involved. The heart is affected in 90% of cases; in 30% of these, cardiac dysfunction is the form of presentation and in 50% it is the cause of death. The commonest form of cardiovascular manifestation is congestive heart failure due to restrictive cardiomyopathy caused by extensive interstitial infiltration of amyloid into the myocardium. Occasionally, it can present as angina, due to infiltration of amyloid into the walls of small vessels. The authors describe the case of a patient in whom the disease presented simultaneously with heart failure and effort angina, as well as intermittent claudication.
Mesquita
Teresa
T
Serviço de Medicina I, Hospital de Egas Moniz, Lisboa, Portugal. teresa.mesquita@sapo.pt
Chorão
Martinha
M
Soares
Isabel
I
Mello e Silva
Alberto
A
Abecasis
Pedro
P
D000368:Aged; D000686:Amyloidosis / Q000150:complications*; D006801:Humans; D007383:Intermittent Claudication / Q000209:etiology*; D008297:Male; D017566:Microvascular Angina / Q000209:etiology*