Primary amyloidosis

Alternative names

Amyloid - primary

Definition

Primary amyloidosis is a disorder in which insoluble protein fibers are deposited in tissues and organs, impairing their function.

Causes, incidence, and risk factors

The cause of primary amyloidosis is unknown, but the condition is related to abnormal production of immunoglobulins by a type of immune cell called plasma cells.

The symptoms depend on the organs affected by the deposits, which can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.

This can result in the following conditions:

• cardiomyopathy (poor heart function)
  
• Kidney failure
  
• Carpal tunnel syndrome (painful swelling of nerves in the wrist)
  
• Malabsorption (inadequate absorption of nutrients from the intestinal tract)
  
• Gastrointestinal reflux (GERD)
  
• Other conditions
  

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. It is related to the malignant plasma cell disorder Multiple Myeloma.

Symptoms

• fatigue
  
• numbness of hands and feet
  
• weak hand grip
  
• Weight loss
  
• Shortness of breath
  
• swelling of the extremities
  
• swallowing difficulties
  
• irregular heart rhythm
  
• skin changes
  
• enlarged tongue
  

Additional symptoms that may be associated with this disease:

• weakness
  
• urine output, decreased