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Chest
Diagnosis
Interstitial, Diffuse, and Inhalational Lung Disease
Metabolic Diseases and Miscellaneous Conditions
Pulmonary Alveolar Proteinosis
Pulmonary Alveolar Proteinosis
Julia Alegría, MD; Jorge Alberto Carrillo-Bayona, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Pulmonary alveolar proteinosis (PAP)

              • Synonyms

                • Pulmonary alveolar lipoproteinosis

              • Definitions

                • PAP: Syndrome of altered surfactant homeostasis characterized by accumulation of surfactant in alveoli and terminal bronchioles
                  • Autoimmune (primary): 90% of cases
                  • Secondary
                  • Hereditary
                  • Congenital

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Carrington JM et al: Pulmonary Alveolar Proteinosis 2021
                                              2. Suzuki T et al: Pulmonary alveolar proteinosis syndrome. Clin Chest Med. 37(3):431-40, 2016
                                              3. Ben-Dov I et al: Autoimmune pulmonary alveolar proteinosis: clinical course and diagnostic criteria. Autoimmun Rev. 13(4-5):513-7, 2014
                                              4. Punatar AD et al: Opportunistic infections in patients with pulmonary alveolar proteinosis. J Infect. 65(2):173-9, 2012
                                              5. Souza CA et al: Comparative study of clinical, pathological and HRCT findings of primary alveolar proteinosis and silicoproteinosis. Eur J Radiol. 81(2):371-8, 2012
                                              6. Ishii H et al: Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis. Chest. 136(5):1348-55, 2009
                                              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

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Pulmonary alveolar proteinosis (PAP)

                                                          • Synonyms

                                                            • Pulmonary alveolar lipoproteinosis

                                                          • Definitions

                                                            • PAP: Syndrome of altered surfactant homeostasis characterized by accumulation of surfactant in alveoli and terminal bronchioles
                                                              • Autoimmune (primary): 90% of cases
                                                              • Secondary
                                                              • Hereditary
                                                              • Congenital

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Carrington JM et al: Pulmonary Alveolar Proteinosis 2021
                                                                                          2. Suzuki T et al: Pulmonary alveolar proteinosis syndrome. Clin Chest Med. 37(3):431-40, 2016
                                                                                          3. Ben-Dov I et al: Autoimmune pulmonary alveolar proteinosis: clinical course and diagnostic criteria. Autoimmun Rev. 13(4-5):513-7, 2014
                                                                                          4. Punatar AD et al: Opportunistic infections in patients with pulmonary alveolar proteinosis. J Infect. 65(2):173-9, 2012
                                                                                          5. Souza CA et al: Comparative study of clinical, pathological and HRCT findings of primary alveolar proteinosis and silicoproteinosis. Eur J Radiol. 81(2):371-8, 2012
                                                                                          6. Ishii H et al: Comparative study of high-resolution CT findings between autoimmune and secondary pulmonary alveolar proteinosis. Chest. 136(5):1348-55, 2009