Secondary amyloidosis (patient information)
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
Overview
Secondary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs especially in the kidney. Clumps of the abnormal proteins are called amyloid deposits.
What are the symptoms of Secondary amyloidosis?
Symptoms depend on the organs affected by the deposits. Since the kidney is the most commonly affected organ, it will manifest with signs and symptoms related to kidney involvement. Symptoms include:
- Frothy urine
- Swelling in the arms and legs
- Periorbital edema
Other less common symptoms include:
- Abdominal mass
- Enlarged tongue
- Fatigue
- Numbness of hands and feet
- Shortness of breath
- Skin changes
- Swallowing problems
- Weak hand grip
- Weight loss
Patients may also have other symptoms due to the underlying disorder, such as:
- Fever
- Joint pain
- Abdominal pain
- Weakness
- Diarrhea
What causes Secondary amyloidosis?
Any condition causing chronic inflammation in the body may cause secondary amyloidosis as well. The most important causes of chronic inflammation include:
- Chronic infections such as:
- Tuberculosis
- Leprosy
- Chronic infection of the bones (Osteomyelitis)
- Chronic infection of the kidney (Chronic pyelonephritis)
- Diseases that lead to chronic states of inflammation include:
- Poorly controlled rheumatoid arthritis)
- Juvenile arthritis
- Ankylosing spondylitis
- Psoriatic arthritis
- Crohn's disease
Secondary amyloidosis can lead to conditions that include:
- Nephrotic syndrome
- Kidney failure
- Liver enlargement
- Neuropathy (nerves that do not work properly)
- Orthostatic hypotension (abnormal drop in blood pressure with standing)
- Carpal tunnel syndrome
- Heart muscle damage (cardiomyopathy) leading to congestive heart failure
- Intestinal malabsorption
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.
Who is at highest risk?
Patients with diseases causing chronic infections and persistent inflammation within the body are at the highest risk of developing secondary amyloidosis.
When to seek urgent medical care?
Call your health care provider if:
- You have symptoms of secondary amyloidosis.
- You know you have secondary amyloidosis and you have difficulty breathing, persistent swelling of the ankles or other areas, decreased urine output, or other symptoms that may indicate complications have developed.
Diagnosis
Your doctor may discover that you have an enlarged liver or spleen. If organ damage is suspected, your doctor may order tests to confirm amyloidosis of that organ. For example:
- Abdominal ultrasound may reveal a swollen liver or spleen.
- An abdominal fat pad aspiration, rectal mucosa biopsy, or bone marrow biopsy can help confirm the diagnosis.
- A heart evaluation, including an ECG, may reveal arrhythmias, abnormal heart sounds, or signs of congestive heart failure. An echocardiogram shows the poor motion of the heart wall, due to a stiff heart muscle.
- A carpal tunnel syndrome evaluation may show that hand grips are weak. Nerve conduction velocity shows abnormalities.
- Kidney function tests may show signs of kidney failure or too much protein in the urine (nephrotic syndrome).
- BUN level is increased.
- Serum creatinine is increased.
- Urinalysis shows protein, casts, or fat bodies.
This disease may also affect the results of the following tests:
- Bence-Jones protein (quantitative)
- Carpal tunnel biopsy
- Gum biopsy
- Immunoelectrophoresis - serum
- Myocardial biopsy
- Nerve biopsy
- Quantitative immunoglobulins
- Tongue biopsy
- Urine protein
Treatment options
In secondary amyloidosis, aggressively treating the disease that is causing the excess amyloid protein can improve symptoms and/or slow the disease from getting worse. Complications such as heart failure, kidney failure, and other problems can sometimes be treated when needed.
Where to find medical care for Secondary amyloidosis?
Directions to Hospitals Treating Secondary amyloidosis
What to expect (Outlook/Prognosis)?
Since kidney is the most commonly involved organ, it may lead to kidney failure and following complications including death.
Possible complications
- Kidney failure
- Abdominal mass
- Numbness in the fingers and toes
- Death
Sources
http://www.nlm.nih.gov/medlineplus/ency/article/000533.htm Template:WH Template:WS CME Category::Cardiology