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Secondary systemic amyloidosis
Definition
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 body-wide. For example, a systemic disease affects the entire body.
See also:
Alternative Names
Amyloidosis - secondary systemic
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
- Hairy cell leukemia
- Hodgkin's disease
- Juvenile chronic arthritis
- Kidney dialysis
- Multiple myeloma
- Reiter syndrome
- Rheumatoid arthritis
- Sjogren syndrome
- Skin abscess or long-term infection
- Systemic lupus erythematosus
- Tuberculosis
Symptoms
- 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.
Possible Complications
- 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's known to increase your risk of this condition, make sure you get it treated. This may help prevent amyloidosis.
References
Buxbaum JN. The amyloidoses. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 296.
Gertz MA, Lacy MQ, Dispenzieri A, Hayman SR. Immunologic light-chain amyloidosis (primary amyloidosis). In: Hoffman R, Benz EJ Jr., Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier; 2008:chap 89.
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, Medical Director, A.D.A.M., Inc.
