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Secondary systemic amyloid

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Contents of this page:

Illustrations

Amyloidosis on the fingers
Amyloidosis on the fingers
Amyloidosis on the face
Amyloidosis on the face
Antibodies
Antibodies

Definition    Return to top

Secondary systemic amyloid is a disorder in which insoluble protein fibers become deposited in tissues and organs impairing their function. It is found in association with chronic infection or chronic inflammatory disease.

Causes, incidence, and risk factors    Return to top

The exact mechanism that causes secondary systemic amyloid is unknown. The risk factors are the presence of chronic inflammatory or infectious diseases. Secondary systemic amyloid occurs in association with multiple myeloma, and chronic conditions (those that last for 5 or more years) such as: rheumatoid arthritis, tuberculosis, long term paraplegia, bronchiectasis, cystic fibrosis, chronic osteomyelitis, recurrent pyogenic (involving pus) skin infection/abscess, decubitus ulcers, chronic renaldialysis, juvenile chronic arthritis, systemic lupus erythematosus, Reiter's syndrome, ankylosing spondylitis, Hodgkin's disease, Sjogren's syndrome, and hairy cell leukemia.

Symptoms are the same as in primary amyloidosis. The symptoms are related to the organs that become affected with the deposits. The affected organs exhibit reduced function.

Symptoms    Return to top

Signs and tests    Return to top

If specific organ damage is suspected, the testing to confirm amyloidosis of that organ may include a biopsy of an affected tissue or organ that is positive for amyloid. Other tests may include: a skin biopsy of subcutaneous fat, a rectal mucosa biopsy, or a bone marrow biopsy. All of these tests are convenient, safe, and likely to be positive for confirmation of the disease.

The physical examination or abdominal ultrasound shows an enlarged liver or spleen.

Cardiac evaluation reveals arrhythmias, abnormal heart sounds, or signs of heart failure:

Renal evaluation reveals renal failure or nephrotic syndrome(excessive protein in the urine):

There may also be an evaluation for carpal tunnel syndrome:

Treatment    Return to top

The treatment is that of the underlying condition to prevent progression of the amyloidosis. There is no specific treatment for the amyloid.

Expectations (prognosis)    Return to top

The severity of the disease depends upon the organs that are affected. Heart and kidney involvement may lead to organ failure and death. Systemic involvement is associated with death in 1 to 3 years.

Complications    Return to top

Calling your health care provider    Return to top

Call for an appointment with your health care provider if you experience symptoms of this condition. Numbness, weak grip, shortness of breath, swelling, bleeding, and irregular heart rhythm are serious symptoms that require prompt intervention.

Prevention    Return to top

There is no known primary prevention. Secondary prevention includes aggressive treatment and control of diseases known to predispose to the development of amyloid.

Update Date: 6/5/2003

Updated by: Ezra E. W. Cohen, M.D., Section of Hematology/Oncology, Department of Medicine, The University of Chicago, Chicago, IL. Review provided by VeriMed Healthcare Network.

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