Definition

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

Secondary means it occurs because of another disease or situation. For example, this condition occurs along with chronic infection or chronic inflammatory disease.

Systemic means body-wide. For example, a systemic disease affects the entire body.

See also:

• Cardiac amyloidosis
• Hereditary amyloidosis
• Primary amyloidosis

Alternative Names

Causes, incidence, and risk factors

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

This condition may occur along with:

• Ankylosing spondylitis
• Bronchiectasis
• Chronic osteomyelitis
• Cystic fibrosis
• Hairy cell leukemia
• Hodgkin's disease
• Long-term skin abscess and certain ulcers
• Long-term kidney dialysis
• Juvenile chronic arthritis
• Multiple myeloma
• Reiter syndrome
• Rheumatoid arthritis
• Sjogren syndrome
• Systemic lupus erythematosus
• Tuberculosis

Symptoms

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

Signs and tests

Tests that may be done include:

• Abdominal ultrasound (may show a swollen liver or spleen)
• Biopsy of skin
• Biopsy of bone marrow
• Blood tests, including creatinineand BUN
• ECG
• Echocardiogram
• 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.

Support Groups

Expectations (prognosis)

How well a person does depends on which organs are affected. If the disease involves the heart and kidneys, it may lead to organ failure and death.

Complications

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

Calling your health care provider

Call your health care provider if you have symptoms of this condition. Numbness, weak grip, shortness of breath, swelling, bleeding, and irregular heart beats are serious symptoms that require prompt medical attention.

Prevention

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

Images

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004.

Kumar V, Abbas AK, Fausto N. Robbins and Cotran Pathologic Basis of Disease. 7th ed. St. Louis, Mo: WB Saunders; 2005.

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