Primary amyloid

Alternative names:

amyloid - primary

Definition:

A disorder of unknown cause, in which insoluble protein-fibers become deposited in tissues and organs, impairing their function.

Causes, incidence, and risk factors:

The cause of primary amyloid is unknown. Symptoms are related to the organs that become affected with the deposits. They occur in the following organs: tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys. Cardiomyopathy, renal failure, carpal tunnel syndrome, malabsorption (inadequate absorption of nutrients from the intestinal tract), gastrointestinal reflux, and other conditions can result. The deposits infiltrate the organs, causing them to lose resilience and become stiff, resulting in barriers to absorption and diffusion of metabolites. Secondary amyloidosis can occur from an infection or existing inflammatory disease.

Risk factors have not been identified. The incidence of primary amyloid is 1 out of 10,000 people.