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{{Secondary amyloidosis (patient information)}}
{{Secondary amyloidosis (patient information)}}


{{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Varun Kumar]], M.B.B.S.
{{CMG}}; {{AE}} {{Sahar}}  '''Associate Editor(s)-In-Chief:''' [[Varun Kumar]], M.B.B.S.


==Overview==
==Overview==
Secondary amyloidosis is a disorder in which abnormal proteins build up in tissues and organs. Clumps of the abnormal proteins are called [[amyloid]] deposits.
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?==
==What are the symptoms of Secondary amyloidosis?==
Symptoms depend on the organs affected by the deposits. These organs can include the [[tongue]], [[intestines]], skeletal and smooth [[muscle]]s, [[nerve]]s, [[skin]], [[ligaments]], [[heart]], [[liver]], [[spleen]], and [[kidneys]].
[[Symptoms]] depend on the organs affected by the deposits. Since the [[kidney]] is the most commonly affected [[Organ (anatomy)|organ]], it will manifest with [[signs]] and [[symptoms]] related to [[kidney]] involvement.
Symptoms include:
 
* Abnormal heart rhythm ([[arrythmia]])
[[Symptoms]] include:
 
* Frothy urine
* [[Swelling]] in the arms and legs
*[[Periorbital edema]]
 
Other less common [[symptoms]] include:
*[[Abdominal mass]]
* [[Enlarged tongue]]
* [[Enlarged tongue]]
* [[Fatigue]]
* [[Fatigue]]
Line 18: Line 25:
* [[Skin changes]]
* [[Skin changes]]
* [[Swallowing]] problems
* [[Swallowing]] problems
* [[Swelling]] in the arms and legs
* Weak hand grip
* Weak hand grip
* [[Weight loss]]
* [[Weight loss]]


Other symptoms that may occur with this disease:
Patients may also have other [[symptoms]] due to the underlying [[disorder]], such as:
* [[Decreased urine output]]
*[[Fever]]
* [[Diarrhea]]
*[[Joint pain]]
* [[Hoarseness]] or changing voice
*[[Abdominal pain]]
* [[Joint pain]]
* [[Weakness]]
* [[Weakness]]
*[[Diarrhea]]


==What causes Secondary amyloidosis?==
==What causes Secondary amyloidosis?==
The cause of secondary [[amyloidosis]] is unknown. The condition is related to abnormal and excess production of [[antibody|antibodies]] by a type of [[immune cell]] called [[plasma cell]]s.
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 (patient information)|Leprosy]]
** Chronic infection of the bones ([[Osteomyelitis (patient information)|Osteomyelitis]])
** Chronic infection of the kidney ([[Chronic pyelonephritis]])
*[[Diseases]] that lead to chronic states of [[inflammation]] include:
** Poorly controlled [[Rheumatoid arthritis (patient information)|rheumatoid arthritis]]
**[[Juvenile arthritis]]
**[[Ankylosing spondylitis (patient information)|Ankylosing spondylitis]]
**[[Psoriatic arthritis (patient information)|Psoriatic arthritis]]
**[[Crohn's disease (patient information)|Crohn's disease]]


Secondary amyloidosis can lead to conditions that include:
Secondary amyloidosis can lead to [[conditions]] that include:
* [[Nephrotic syndrome]]
* [[Kidney failure]]
* [[Hepatomegaly|Liver enlargement]]
* [[Neuropathy]] (nerves that do not work properly)
* [[Orthostatic hypotension]] (abnormal drop in [[blood pressure]] with standing)
* [[Carpal tunnel syndrome]]
* [[Carpal tunnel syndrome]]
* Heart muscle damage ([[cardiomyopathy]]) leading to [[congestive heart failure]]
* Heart muscle damage ([[cardiomyopathy]]) leading to [[congestive heart failure]]
* Intestinal [[malabsorption]]
* Intestinal [[malabsorption]]
* [[Hepatomegaly|Liver enlargement]]
* [[Kidney failure]]
* [[Nephrotic syndrome]]
* [[Neuropathy]] (nerves that do not work properly)
* [[Orthostatic hypotension]] (abnormal drop in [[blood pressure]] with standing)
The deposits build up in the affected organs, causing them to become stiff, which decreases their ability to function.


The deposits build up in the affected [[organs]], causing them to become stiff, which decreases their ability to function.
==Who is at highest risk?==
==Who is at highest risk?==
Risk factors have not been identified. secondary [[amyloidosis]] is rare. It is similar to [[multiple myeloma]], and is treated the same way.
[[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?==
==When to seek urgent medical care?==
Call your health care provider if:
Call your health care provider if:
*You have symptoms of secondary [[amyloidosis]].
*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.
*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 [[Complication (medicine)|complications]] have developed.
 
==Diagnosis==
==Diagnosis==
Your doctor may discover that you have an enlarged [[liver]] or [[spleen]].
A [[patient]] with a chronic [[Inflammatory diseases|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 (patient information)|nephrotic syndrome]], or [[Renal failure|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]].


If organ damage is suspected, your doctor may order tests to confirm [[amyloidosis]] of that organ. For example:
Once AA amyloidosis is confirmed it is important to look for the primary underlying [[Inflammatory disease|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]].
*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.
*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 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:
This [[disease]] may also affect the results of the following tests:
* [[Bence-Jones protein]] (quantitative)
* Carpal tunnel [[biopsy]]
* Carpal tunnel [[biopsy]]
* Gum biopsy
* Gum biopsy
* [[Immunoelectrophoresis]] - serum
* [[Myocardial biopsy]]
* [[Myocardial biopsy]]
* [[Nerve biopsy]]
* [[Nerve biopsy]]
* Quantitative [[immunoglobulins]]
* Tongue biopsy
* Tongue biopsy
* Urine protein


==Treatment options==
==Treatment options==
Some patients with secondary [[amyloidosis]] respond to [[chemotherapy]] focused on the abnormal [[plasma cell]]s. A [[stem cell transplant]] may be done, as in [[multiple myeloma]].
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.
 
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?==
==Where to find medical care for Secondary amyloidosis?==
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|Secondary amyloidosis}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed    Directions to Hospitals Treating Secondary amyloidosis]
[http://maps.google.com/maps?f=q&hl=en&geocode=&q={{urlencode:{{#if:{{{1|}}}|{{{1}}}|Secondary amyloidosis}}}}&sll=37.0625,-95.677068&sspn=65.008093,112.148438&ie=UTF8&ll=37.0625,-95.677068&spn=91.690419,149.414063&z=2&source=embed    Directions to Hospitals Treating Secondary amyloidosis]
==What to expect (Outlook/Prognosis)?==
==What to expect (Outlook/Prognosis)?==
How severe the disease is depends on the organs that are affected. When the [[heart]] and [[kidney]] are involved, it may lead to organ failure and death. Body-wide (systemic) involvement can lead to death in 1 to 3 years.
Since [[kidney]] is the most commonly involved organ, it may lead to [[kidney failure]] and other [[complications]] including death.
 
==Possible complications==
==Possible complications==
*[[Congestive heart failure (patient information)|Congestive heart failure]]
*[[Endocrine diseases|Endocrine failure]] (hormonal disorder)
*[[Kidney failure]]
*[[Kidney failure]]
*[[Respiratory failure]]
*[[Abdominal mass]]
*[[Numbness]] in the fingers and toes
*[[Death]]
*[[Death]]



Latest revision as of 18:21, 7 November 2019

For the WikiDoc page for this topic, click here

Secondary amyloidosis

Overview

What are the symptoms?

What are the causes?

Who is at highest risk?

When to seek urgent medical care?

Diagnosis

Treatment options

Where to find medical care for Secondary amyloidosis?

What to expect (Outlook/Prognosis)?

Possible complications

Secondary amyloidosis On the Web

Ongoing Trials at Clinical Trials.gov

Images of Secondary amyloidosis

Videos on Secondary amyloidosis

FDA on Secondary amyloidosis

CDC on Secondary amyloidosis

Secondary amyloidosis in the news

Blogs on Secondary amyloidosis

Directions to Hospitals Treating Secondary amyloidosis

Risk calculators and risk factors for Secondary amyloidosis

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:

Other less common symptoms include:

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:

Secondary amyloidosis can lead to conditions that include:

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:

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:

This disease may also affect the results of the following tests:

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

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/000533.htm Template:WH Template:WS CME Category::Cardiology