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 due to long-term (chronic) infection or inflammation. In contrast, primary amyloidosis means there is no other disease that is causing the condition.

Systemic means that the disease affects the entire body.

Alternative Names

Amyloidosis - secondary systemic; AA amyloidosis

Causes

The exact cause of secondary systemic 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 in the spine
Bronchiectasis -- disease in which the large airways in the lungs are damaged by chronic infection
Chronic osteomyelitis -- bone infection
Cystic fibrosis -- a genetic disease that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body, leading to chronic infection of the lungs
Familial Mediterranean fever -- inherited disorder of repeated fevers and inflammation that often affects the lining of the abdomen, chest, or joints
Gout
Hairy cell leukemia -- a type of blood cancer
Hodgkin disease -- cancer of the lymph tissue
Inflammatory bowel disease
Juvenile idiopathic arthritis -- arthritis that affects children
Multiple myeloma -- a type of blood cancer
Psoriatic arthritis
Reactive arthritis -- a group of conditions that causes swelling and inflammation of the joints, eyes, and urinary and genital systems)
Rheumatoid arthritis
Systemic lupus erythematosus -- an autoimmune disorder
Tuberculosis
Vasculitis (inflammation of the blood vessels)

Symptoms

Symptoms of secondary systemic amyloidosis depend on which body tissue is affected by the protein deposits. These deposits damage normal tissues. This may lead to the symptoms or signs of this illness, including:

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 (blood urea nitrogen)
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 an immunosuppressive drug (medicine that treats the immune system) is prescribed.

Outlook (Prognosis)

How well a person does depends on which organs are affected. It also depends on, 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:

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.

Gallery

Amyloidosis of the fingers — 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.

References

Gertz MA. Amyloidosis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 179.

Papa R, Lachmann HJ. Secondary, AA, amyloidosis. Rheum Dis Clin North Am. 2018;44(4):585-603. PMID: 30274625 pubmed.ncbi.nlm.nih.gov/30274625/.

Last reviewed April 24, 2021 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, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team..

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