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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 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
  • 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

 

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

 

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

 

 

Open References

References

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

 
  • 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: 5/3/2015

        Reviewed By: Laura J. Martin, MD, MPH, ABIM Board Certified in Internal Medicine and Hospice and Palliative Medicine. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, 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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