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Primary amyloidosis    See images

Overview | Symptoms | Treatment | Prevention

Alternative names:

Amyloid - primary

Definition:

A disorder in which insoluble protein fibers are deposited in tissues and organs, impairing their function.

Causes and Risks

The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of immunoglobulins by plasma cells (a type of immune cell). Symptoms are related to the organs affected by the deposits, which can include the following: tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys. Cardiomyopathy (decreased heart function), renal failure (kidney failure), carpal tunnel syndrome (painful swelling of nerves in the wrist), malabsorption (inadequate absorption of nutrients from the intestinal tract), gastrointestinal reflux, and other conditions can result. The deposits infiltrate the affected organs, causing them to lose resilience and become stiff, which decreases their ability to function. Secondary amyloidosis can be caused by infection, inflammatory diseases, and sometimes cancer.

Risk factors have not been identified. Primary amyloidosis is rare, occurring only in 1 out of 100,000 people.

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