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Types of amyloidosis

Primary systemic amyloidosis usually develops between the ages of 50 and 60. With about 2,000 new cases diagnosed annually, primary systemic amyloidosis is the most common form of this disease in the United States. Also known as light-chain-related amyloidosis, it may also occur in association with multiple myeloma (bone marrow cancer).

Secondary amyloidosis is a result of chronic infection or inflammatory disease. It is often associated with:

• familial Mediterranean fever (a bacterial infection characterized by chills, weakness, headache, and recurring fever)
• granulomatous ileitis (inflammation of the small intestine)
• Hodgkin's disease (cancer of the lymphatic system)
• leprosy
• osteomyelitits (bacterial infection of bone and bone marrow)
• rheumatoid arthritis

Familial or hereditary amyloidosis is the only inherited form of the disease. It occurs in members of most ethnic groups, and each family has a distinctive pattern of symptoms and organ involvement. Hereditary amyloidosis is though to be autosomal dominant, which means that only one copy of the defective gene is necessary to cause the disease. A child of a parent with familial amyloidosis has a 50-50 chance of developing the disease.
Amyloidosis can involve any organ or system in the body. The heart, kidneys, gastrointestinal system, and nervous system are affected most often. Other common sites of amyloid accumulation include the brain, joints, liver, spleen, pancreas, respiratory system, and skin.