Secondary amyloidosis (patient information)
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Secondary amyloidosis |
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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] Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S.
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:
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:
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 kidney dysfunction, including periorbital edema, swelling of the ankles or others area, abdominal mass and etc.
- 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
A patient with a chronic inflammatory disease or chronic infection may develop high levels of protein in the urine or other associated symptoms. These patients should be tested for AA amyloid deposition. When renal damage occurs, it can be clinically shown as proteinuria (the protein found in the urine), nephrotic syndrome, or impairment of renal (kidney) function.
- A 24-hour urine collection should be performed to look at the level of protein in the patient’s urine sample.
- A biopsy of renal (kidney) tissue is recommended to get an accurate diagnosis.
- Congo-red staining of the sample is required to confirm the diagnosis. An apple green color under a ‘polarizing’ microscope is consistent with the diagnosis.
Once AA amyloidosis is confirmed it is important to look for the primary underlying inflammatory condition, if not already known. Then, the next step is to determine the extent of amyloid involvement in all organs and develop a plan for treatment. This is done by a careful physical examination and laboratory studies of kidney and heart function. Your doctor may order tests to confirm amyloidosis of other 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.
This disease may also affect the results of the following tests:
- Carpal tunnel biopsy
- Gum biopsy
- Myocardial biopsy
- Nerve biopsy
- Tongue biopsy
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 other 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