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Pulmonary Amyloidosis
Laura E. Heyneman, MD
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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

                                            1. Stern LK et al: Updates in cardiac amyloidosis diagnosis and treatment. Curr Oncol Rep. 23(4):47, 2021
                                            2. Wu CW et al: Sjögren's syndrome with nodular pulmonary amyloidosis. Respirol Case Rep. 9(1):e00699, 2021
                                            3. 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
                                            4. Milani P et al: The lung in amyloidosis. Eur Respir Rev. 26(145), 2017
                                            5. Schelbert EB et al: State of the art: clinical applications of cardiac T1 mapping. Radiology. 278(3):658-76, 2016
                                            6. Wechalekar AD et al: Systemic amyloidosis. Lancet. 387(10038):2641-54, 2016
                                            7. Czeyda-Pommersheim F et al: Amyloidosis: modern cross-sectional imaging. Radiographics. 35(5):1381-92, 2015
                                            8. Park MA et al: 18F-florbetapir binds specifically to myocardial light chain and transthyretin amyloid deposits: autoradiography study. Circ Cardiovasc Imaging. 8(8), 2015
                                            9. Baqir M et al: Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respir Med. 107(4):616-21, 2013
                                            10. De Smet K et al: MRI in the assessment of non ischemic myocardial diseases. Eur J Radiol. 81(7):1546-8, 2012
                                            11. 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

                                                                                      1. Stern LK et al: Updates in cardiac amyloidosis diagnosis and treatment. Curr Oncol Rep. 23(4):47, 2021
                                                                                      2. Wu CW et al: Sjögren's syndrome with nodular pulmonary amyloidosis. Respirol Case Rep. 9(1):e00699, 2021
                                                                                      3. 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
                                                                                      4. Milani P et al: The lung in amyloidosis. Eur Respir Rev. 26(145), 2017
                                                                                      5. Schelbert EB et al: State of the art: clinical applications of cardiac T1 mapping. Radiology. 278(3):658-76, 2016
                                                                                      6. Wechalekar AD et al: Systemic amyloidosis. Lancet. 387(10038):2641-54, 2016
                                                                                      7. Czeyda-Pommersheim F et al: Amyloidosis: modern cross-sectional imaging. Radiographics. 35(5):1381-92, 2015
                                                                                      8. Park MA et al: 18F-florbetapir binds specifically to myocardial light chain and transthyretin amyloid deposits: autoradiography study. Circ Cardiovasc Imaging. 8(8), 2015
                                                                                      9. Baqir M et al: Amyloid-associated cystic lung disease in primary Sjögren's syndrome. Respir Med. 107(4):616-21, 2013
                                                                                      10. De Smet K et al: MRI in the assessment of non ischemic myocardial diseases. Eur J Radiol. 81(7):1546-8, 2012
                                                                                      11. Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010