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| __NOTOC__ | | __NOTOC__ |
| {{Infobox_Disease |
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| Name = {{PAGENAME}} |
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| Image = Small bowel duodenum with amyloid deposition congo red 10X.jpg |
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| Caption = Small bowel duodenum with amyloid deposition congo red 10X |
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| DiseasesDB = 633 |
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| ICD10 = {{ICD10|E|85||e|70}} |
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| ICD9 = {{ICD9|277.3}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = |
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| MeshID = D000686 |
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| }}
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| {{Amyloidosis}} | | {{Amyloidosis}} |
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| '''For patient information, click [[Amyloidosis (patient information)|here]] | | '''For patient information, click [[Amyloidosis (patient information)|here]]''' |
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| {{CMG}}; {{AE}} {{RT}} | | {{CMG}}; {{AE}}{{Sab}}, {{HK}}, {{SHH}} |
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| {{SK}} Amyloid disease | | {{SK}} |
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| ==[[Amyloidosis overview|Overview]]== | | ==[[Amyloidosis overview|Overview]]== |
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| ==[[Amyloidosis causes|Causes]]== | | ==[[Amyloidosis causes|Causes]]== |
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| ==[[Amyloidosis differential diagnosis|Differentiating Amyloidosis from other Diseases]]== | | ==[[Amyloidosis differential diagnosis|Differentiating Amyloidosis from other Diseases]]== |
| Amyloidosis should be differentiated from the following systemic diseases:
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| * Acute [[myocarditis]]
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| * [[Bechterew's Disease]]
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| * [[Bronchiectasis]]
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| * [[Carpal Tunnel Syndrome]]
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| * [[Collagen Vascular Disease]]
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| * [[Drug]]/[[toxic]] [[nephropathy]]
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| * [[Familial Mediterranean Fever]]
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| * [[Glomerulonephritis]]
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| * Hemodialysis [[Amyloidosis]]
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| * Interstitial lung diseases
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| * [[Leprosy]]
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| * Monoclonal gammopathies
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| * [[Multiple Myeloma]]
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| * Myocardial fibrosis
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| * [[Nephrotic Syndrome]]
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| * [[Osteomyelitis]]
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| * [[Peripheral neuropathy]]
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| * Restrictive [[cardiomyopathy]]
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| * [[Rheumatoid Arthritis]]
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| * [[Rheumatoid Polyarteritis]]
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| * [[Syphilis]]
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| * [[Systemic Lupus Erythematosus]]
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| * [[Tuberculosis]]
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| * [[Ulcerative colitis]]
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| * Vitamin deficiencies
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| ==[[Amyloidosis epidemiology and demographics|Epidemiology and Demographics]]== | | ==[[Amyloidosis epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[Amyloidosis risk factors|Risk Factors]]== | | ==[[Amyloidosis risk factors|Risk Factors]]== |
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| | ==[[Amyloidosis screening|Screening]]== |
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| ==[[Amyloidosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[Amyloidosis natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| ===Complications===
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| Possible complications of amyloidosis include:
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| * Hyposplenism
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| * [[Malabsorption syndrome]]
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| * [[Myopathy]]
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| * [[Proximal renal tubular acidosis]]
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| * [[Renal failure]]
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| * Vascular hemostatic disorder
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| * Chronic brain failure
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| * [[Nephrotic syndrome]]
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| * [[Myocarditis]]
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| * [[Restrictive cardiomyopathy]]
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| * [[Pulmonary fibrosis]]
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| * [[Autonomic neuropathy]]
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| * [[Pericardial effusion]]
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| * [[Congestive heart failure (patient information)|Congestive heart failure]]
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| * [[Endocrine failure]] (hormonal disorder)
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| * [[Respiratory failure]]
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| * [[Hypogonadic hypogonadism]]
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| * [[Death]]
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| ===Prognosis===
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| The severity the disease 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.
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| ==Diagnosis== | | ==Diagnosis== |
| [[Amyloidosis history and symptoms|History and Symptoms]] | [[Amyloidosis physical examination|Physical Examination]] | [[Amyloidosis laboratory findings|Laboratory Findings]] | [[Amyloidosis electrocardiogram|Electrocardiogram]] | [[Amyloidosis x ray|X Ray]] | [[Amyloidosis CT|CT]] | [[Amyloidosis MRI|MRI]] | [[Amyloidosis other imaging findings|Other Imaging Findings]] | | [[Amyloidosis diagnostic study of choice|Diagnostic study of choice]] | [[Amyloidosis history and symptoms|History and Symptoms]] | [[Amyloidosis physical examination|Physical Examination]] | [[Amyloidosis laboratory findings|Laboratory Findings]] | [[Amyloidosis electrocardiogram|Electrocardiogram]] | [[Amyloidosis x ray|X-ray]] | [[Amyloidosis echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Amyloidosis CT scan|CT scan]] | [[Amyloidosis MRI|MRI]] | [[Amyloidosis other imaging findings|Other Imaging Findings]] | [[Amyloidosis other diagnostic studies|Other Diagnostic Studies]] |
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| Symptoms depend on the organs affected by the deposits. These organs can include the tongue, intestines, skeletal and smooth muscles, nerves, skin, ligaments, heart, liver, spleen, and kidneys.
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| Symptoms include:
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| * Palpitations
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| * [[Hemoptysis]]
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| * [[Cough]]
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| * [[Lightheadedness]]
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| * [[Fatigue]]
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| * [[Numbness]] of hands and feet
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| * [[Shortness of breath]]
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| * Skin changes
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| * Swallowing problems
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| * Swelling in the arms and legs
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| * Weak hand grip
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| * [[Weight loss]]
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| Other symptoms that may occur with this disease:
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| * [[Decreased urine output]]
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| * [[Diarrhea]]
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| * [[Hoarseness]] or changing voice
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| * [[Joint pain]]
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| * [[Weakness]]
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| ===Physical Examination===
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| ====Vitals====
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| * [[Hypertension]]
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| * [[Tachycardia]]
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| ====Skin====
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| * The most common skin findings are
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| ** [[Petechiae]] (small red blood spots).
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| ** [[Ecchymoses]] (small, flat blood spots that are round or irregular shape and bluish/purplish in colour)
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| ** Waxy papular nodules can be seen around the eye lids, neck and also in the groin.
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| ** Brittle nails.
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| ** Edema due to [[heart failure]]
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| ** Patches of [[hair loss]] can be seen.
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| ====Head====
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| * [[Parotid gland]] enlargement
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| * Submandibular gland enlargement
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| ====Eye====
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| * Raised intraocular pressure may be present.
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| ====Throat====
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| * [[Enlarged tongue]]
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| ====Abdomen====
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| * [[Hepatomegaly]]
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| ====Extremities====
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| * [[Carpal tunnel syndrome]]
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| * [[Numbness]] of the fingers and toes
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| * [[Raynaud phenomenon]]
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| ===Laboratory Findings===
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| * [[Complete blood count]] - may reveal [[anemia]].
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| * [[Erythrocyte sedimentation rate]] - elevated
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| * Liver function tests
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| ** Serum [[albumin]]
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| ** Alkaline phosphatase
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| ** [[Bilirubin]], total
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| * Kidney function tests may show signs of kidney failure or too much protein in the urine ([[nephrotic syndrome]]).
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| ** [[BUN]] level is increased.
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| ** Serum [[creatinine]] is increased.
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| ** Urinalysis shows protein, casts, or fat bodies.
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| ** [[Bence-Jones protein]] (quantitative)
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| * Serum [[troponin]], [[B-type natriuretic peptide]], and [[beta-2-microglobulin]] - prognostic markers for [[heart failure]].
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| * [[Immunoelectrophoresis]] - serum
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| * Amyloid deposits can be identified [[histologically]] by [[Congo red]] staining and viewing under [[polarized light]] where amyloid deposits produce a distinctive 'apple green birefringence'. Alternatively, [[Thioflavin|thioflavin T]] stain may be used
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| * Immunohistochemical studies on the amyloid deposits - to differentiate from different types of amyloid.
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| * [[Biopsy]] - is needed depending on the involvement of the specific organ. e.g [[tongue]], [[myocardium]], [[skin]]. An abdominal fat pad aspiration, rectal mucosa biopsy, or bone marrow biopsy can help confirm the diagnosis. They reveal positive findings in 80% patients.
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| ===Electrocardiogram===
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| * Abnormal rhythm - [[arrhythmias]]
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| * [[Low voltage recordings]]
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| ===Imaging===
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| ====X-ray====
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| * Coin lesion (chest x-ray)
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| ====Echocardiogram====
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| * [[Echocardiogram]] is a valuable tool in the evaluation of the extent of heart involvement in [[amyloidosis]]. The common findings include:
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| ** Thickened ventricular wall
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| ** Ventricular wall motion abnormalities
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| ====CT====
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| '''Gastrointestinal amyloidosis on imaging'''
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| <gallery>
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| Image:
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| Bowel-amyloidosis-001.jpg
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| Image:
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| Bowel-amyloidosis-002.jpg
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| Image:
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| Bowel-amyloidosis-003.jpg
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| Image:
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| Bowel-amyloidosis-004.jpg
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| </gallery>
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| ====MRI====
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| * Cardiac [[MRI]] is used when [[echocardiogram]] fails to differentiate amyloidosis from [[hypertrophic cardiomyopathy]].
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| ====Other Imaging Studies====
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| * In addition, all amyloid deposits contain [[serum amyloid P component]] (SAP), a circulating protein of the [[pentraxin]] family. [[Radionuclide]] SAP scans have been developed which can anatomically localize amyloid deposits in patients.
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| * Tissue Doppler and myocardial strain rate imaging has been proven to be very sensitive for the assessment of myocardial dysfunction in [[restrictive cardiomyopathy]]. Doppler studies may show abnormal wall motion early in the disease process.
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| ==Treatment== | | ==Treatment== |
| [[Amyloidosis medical therapy|Medical Therapy]] | | [[Amyloidosis medical therapy|Medical Therapy]] | [[Amyloidosis surgery|Surgery]] | [[Amyloidosis primary prevention|Primary Prevention]] | [[Amyloidosis secondary prevention|Secondary Prevention]] | [[Amyloidosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Amyloidosis future or investigational therapies|Future or Investigational Therapies]] |
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| There is no treatment for primary amyloidosis.
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| * The initial target in the treatment of this disorder is to correct the organ failure, as the disease is discovered at an advanced stage.
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| ** [[Nephrotic syndrome]] is treated using supportive therapy and [[diuretics]].
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| ** [[Renal failure]] is treated with [[dialysis]]
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| ** [[Heart failure]] is treated using [[diuretics]]
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| ** Gastrointestinal and nerve involvement are treated symptomatically.
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| Treatment options with limited success include [[melphalan]] and [[prednisone]], and [[colchicine]].
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| ==References==
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| {{reflist|2}}
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| {{Metabolic pathology}}
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| [[de:Amyloidose]]
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| [[et:Amüloidoos]]
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| [[ja:アミロイドーシス]]
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| [[ru:Амилоидоз]]
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| [[fi:Amyloidoosi]]
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| [[zh:類澱粉沉著症]]
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| {{WikiDoc Help Menu}}
| | ==Case Studies== |
| {{WikiDoc Sources}}
| | [[Amyloidosis case study one|Case #1]] |
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| [[Category:Disease]] | | [[Category:Disease]] |
| [[Category:Rheumatology]]
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| [[Category:Cardiology]] | | [[Category:Cardiology]] |
| [[Category:Mature chapter]] | | [[Category:Pulmonology]] |
| [[Category:Metabolic disorders]] | | [[Category:Immunology]] |
| [[Category:Inborn errors of metabolism]]
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| [[Category:Endocrinology]]
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