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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Amyloid light chain (AL)
Serum amyloid A (AA)
Transthyretin amyloid (ATTR)
Amyloid β-2 microglobulin (Aβ2M)
Wild-type transthyretin amyloid (ATTRwt)
Lysozyme amyloid (ALys)
Gelsolin amyloid (AGel)
Definitions
Deposition of abnormal insoluble proteins within tissue
Localized vs. systemic
Localized disease or disease manifesting primarily in one organ vs. systemic disease
Localized to respiratory tract: Primarily AL
Systemic: Associated with AL, AA, ATTR, etc.
Primary: Associated with primary cell dyscrasias
Secondary: Associated with chronic infections or inflammatory disorders
Systemic AL: Formerly known as primary amyloidosis
Terms primary and secondary amyloidosis refer to chemical make-up of abnormalities
Primary: i.e., AL amyloidosis
Secondary: i.e., AA amyloidosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Stern LK et al: Updates in cardiac amyloidosis diagnosis and treatment. Curr Oncol Rep. 23(4):47, 2021
Wu CW et al: Sjögren's syndrome with nodular pulmonary amyloidosis. Respirol Case Rep. 9(1):e00699, 2021
Brandelik SC et al: CT features in amyloidosis of the respiratory system - comprehensive analysis in a tertiary referral center cohort. Eur J Radiol. 129:109123, 2020
Milani P et al: The lung in amyloidosis. Eur Respir Rev. 26(145), 2017
Schelbert EB et al: State of the art: clinical applications of cardiac T1 mapping. Radiology. 278(3):658-76, 2016
Wechalekar AD et al: Systemic amyloidosis. Lancet. 387(10038):2641-54, 2016
Czeyda-Pommersheim F et al: Amyloidosis: modern cross-sectional imaging. Radiographics. 35(5):1381-92, 2015
Park MA et al: 18F-florbetapir binds specifically to myocardial light chain and transthyretin amyloid deposits: autoradiography study. Circ Cardiovasc Imaging. 8(8), 2015
Baqir M et al: Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respir Med. 107(4):616-21, 2013
De Smet K et al: MRI in the assessment of non ischemic myocardial diseases. Eur J Radiol. 81(7):1546-8, 2012
Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Amyloid light chain (AL)
Serum amyloid A (AA)
Transthyretin amyloid (ATTR)
Amyloid β-2 microglobulin (Aβ2M)
Wild-type transthyretin amyloid (ATTRwt)
Lysozyme amyloid (ALys)
Gelsolin amyloid (AGel)
Definitions
Deposition of abnormal insoluble proteins within tissue
Localized vs. systemic
Localized disease or disease manifesting primarily in one organ vs. systemic disease
Localized to respiratory tract: Primarily AL
Systemic: Associated with AL, AA, ATTR, etc.
Primary: Associated with primary cell dyscrasias
Secondary: Associated with chronic infections or inflammatory disorders
Systemic AL: Formerly known as primary amyloidosis
Terms primary and secondary amyloidosis refer to chemical make-up of abnormalities
Primary: i.e., AL amyloidosis
Secondary: i.e., AA amyloidosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Stern LK et al: Updates in cardiac amyloidosis diagnosis and treatment. Curr Oncol Rep. 23(4):47, 2021
Wu CW et al: Sjögren's syndrome with nodular pulmonary amyloidosis. Respirol Case Rep. 9(1):e00699, 2021
Brandelik SC et al: CT features in amyloidosis of the respiratory system - comprehensive analysis in a tertiary referral center cohort. Eur J Radiol. 129:109123, 2020
Milani P et al: The lung in amyloidosis. Eur Respir Rev. 26(145), 2017
Schelbert EB et al: State of the art: clinical applications of cardiac T1 mapping. Radiology. 278(3):658-76, 2016
Wechalekar AD et al: Systemic amyloidosis. Lancet. 387(10038):2641-54, 2016
Czeyda-Pommersheim F et al: Amyloidosis: modern cross-sectional imaging. Radiographics. 35(5):1381-92, 2015
Park MA et al: 18F-florbetapir binds specifically to myocardial light chain and transthyretin amyloid deposits: autoradiography study. Circ Cardiovasc Imaging. 8(8), 2015
Baqir M et al: Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respir Med. 107(4):616-21, 2013
De Smet K et al: MRI in the assessment of non ischemic myocardial diseases. Eur J Radiol. 81(7):1546-8, 2012
Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
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