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    Secondary Systemic Amyloidosis

    Amyloidosis - secondary systemic

    Secondary systemic amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called amyloid deposits.

    Secondary means it occurs because of another disease or situation. For example, this condition usually occurs as a result of chronic infection or chronic inflammatory disease. Primary amyloidosis means there is no disease that is causing the condition.

    Systemic meansthat the diseaseaffects the entire body.

    Causes

    The exact cause of amyloidosis is unknown. You are more likely to develop secondary systemic amyloidosis if you have a long-term infection or inflammation.

    This condition may occur with:

    • Ankylosing spondylitis
    • Bronchiectasis
    • Chronic osteomyelitis
    • Cystic fibrosis
    • Familial Mediterranean fever
    • Hairy cell leukemia
    • Hodgkin disease
    • Juvenile chronic arthritis
    • Kidney dialysis
    • Multiple myeloma
    • Reiter syndrome
    • Rheumatoid arthritis
    • Sjogren syndrome
    • Systemic lupus erythematosus
    • Tuberculosis

    Symptoms

    The symptoms of secondary systemic amyloidosis depend on which body tissue is affected by the protein deposits. These deposits damage normal tissues, leading to the symptomsor signs of this illness:

    • Bleeding in the skin
    • Fatigue
    • Irregular heartbeat
    • Numbness of hands and feet
    • Rash
    • Shortness of breath
    • Swallowing difficulties
    • Swollen arms or legs
    • Swollen tongue
    • Weak hand grip
    • Weight loss

    Exams and Tests

    Tests that may be done include:

    • Abdominal ultrasound (may show a swollen liver or spleen)
    • Biopsy or aspiration of fat just beneath the skin (subcutaneous fat)
    • Biopsy of rectum
    • Biopsy of skin
    • Biopsy of bone marrow
    • Blood tests, including creatinine and BUN
    • Echocardiogram
    • Electrocardiogram (ECG)
    • Nerve conduction velocity
    • Urinalysis

    Treatment

    The condition that is causing the amyloidosis should be treated. Some persons may need a bone marrow or stem cell transplant. There is no specific treatment for amyloidosis itself.

    Outlook (Prognosis)

    How well a person does depends on which organs are affected and whether the disease that is causing it can be controlled. If the disease involves the heart and kidneys, it may lead to organ failure and death.

    • Endocrine failure
    • Heart failure
    • Kidney failure
    • Respiratory failure

    When to Contact a Medical Professional

    Call your health care provider if you have symptoms of this condition. The following are serious symptoms that need prompt medical attention:

    • Bleeding
    • Irregular heartbeat
    • Numbness
    • Shortness of breath
    • Swelling
    • Weak grip

    Prevention

    If you have a disease that isknown to increase your risk of this condition, make sure you get it treated. This may help prevent amyloidosis.

    References

    Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman’s Cecil Medicine. 24th ed. Philadelphia, Pa.: Elsevier Saunders; 2011:chap 194.

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      • Amyloidosis on the face

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      A Closer Look

      Self Care

        Tests for Secondary Systemic Amyloidosis

          Review Date: 4/14/2013

          Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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