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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 long-term (chronic) infection or chronic inflammatory disease. Primary amyloidosis means there is no other disease that is causing the condition.

Systemic means that the disease affects 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 -- a form of arthritis that mostly affects the bones and joints at the base of the spine
  • Bronchiectasis -- disease in which the large airways in the lungs are damaged
  • Chronic osteomyelitis -- bone infection
  • Cystic fibrosis -- disease that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body
  • Familial Mediterranean fever -- inherited disorder of repeated fevers and inflammation that often affects the lining of the abdomen, chest, or joints
  • Hairy cell leukemia -- a type of blood cancer
  • Hodgkin disease -- cancer of the lymph tissue
  • Juvenile chronic arthritis -- arthritis that affects children
  • Kidney dialysis
  • Multiple myeloma -- a type of blood cancer
  • Reiter syndrome -- a group of conditions that causes swelling and inflammation of the joints, eyes, and urinary and genital systems)
  • Rheumatoid arthritis
  • Sjögren syndrome -- an autoimmune disorder in which the glands that produce tears and saliva are destroyed
  • Systemic lupus erythematosus -- an autoimmune disorder
  • Tuberculosis

 

Symptoms

 

Symptoms of secondary systemic amyloidosis depend on which body tissue is affected by the protein deposits. These deposits damage normal tissues, leading to the symptoms or 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

 

The health care provider will perform a physical examination and ask about your symptoms.

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. In some cases, the drug colchicine or a biologic drug (medicine that treats the immune system) is prescribed.

 

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.

 

Possible Complications

 

Health problems that may result from secondary systemic amyloidosis include:

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

 

When to Contact a Medical Professional

 

Call your 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 is known to increase your risk for this condition, make sure you get it treated. This may help prevent amyloidosis.

 

 

References

Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 188.

Text only

 
  • Amyloidosis on the fingers

    Amyloidosis on the fingers - illustration

    Amyloidosis refers to the extracellular deposition of a protein called amyloid. This protein deposition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging, or may occur with several other conditions. In this picture, we see how amyloidosis can affect the skin as nodular deposits on the fingers.

    Amyloidosis on the fingers

    illustration

  • Amyloidosis on the face

    Amyloidosis on the face - illustration

    Amyloidosis refers to deposits of a protein (called amyloid) in the tissues. This condition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging. In this picture, we see how amyloidosis can cause a patchy, bruised appearance to the skin. Bruises of the skin around the eyes are referred to as the characteristic "pinched purpura".

    Amyloidosis on the face

    illustration

  • Antibodies

    Antibodies - illustration

    Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

    Antibodies

    illustration

    • Amyloidosis on the fingers

      Amyloidosis on the fingers - illustration

      Amyloidosis refers to the extracellular deposition of a protein called amyloid. This protein deposition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging, or may occur with several other conditions. In this picture, we see how amyloidosis can affect the skin as nodular deposits on the fingers.

      Amyloidosis on the fingers

      illustration

    • Amyloidosis on the face

      Amyloidosis on the face - illustration

      Amyloidosis refers to deposits of a protein (called amyloid) in the tissues. This condition can affect multiple organs. The deposition of amyloid may be a by-product of normal aging. In this picture, we see how amyloidosis can cause a patchy, bruised appearance to the skin. Bruises of the skin around the eyes are referred to as the characteristic "pinched purpura".

      Amyloidosis on the face

      illustration

    • Antibodies

      Antibodies - illustration

      Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

      Antibodies

      illustration

    A Closer Look

     

    Self Care

     

      Tests for Secondary systemic amyloidosis

       
         

        Review Date: 5/21/2017

        Reviewed By: Laura J. Martin, MD, MPH, ABIM Board Certified in Internal Medicine and Hospice and Palliative Medicine, Atlanta, GA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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