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Pleuropulmonary Fibroelastosis
Takeshi Johkoh, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Pleuroparenchymal fibroelastosis (PPFE)
            • Synonyms

              • Amitani disease
            • Definitions

              • Rare interstitial pneumonia characterized by upper lobe-predominant elastotic fibrosis involving pleura and subpleural lung parenchyma
                • Idiopathic or secondary (e.g., late complication of lung or hematopoietic stem cell transplantation)

            IMAGING

            • Radiographic Findings

              • CT Findings

                • Nuclear Medicine Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Mariani F et al: Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagn Interv Radiol. 22(5):400-6, 2016
                                    2. Johkoh T et al: Rare idiopathic intestinal pneumonias (IIPs) and histologic patterns in new ATS/ERS multidisciplinary classification of the IIPs. Eur J Radiol. 84(3):542-6, 2015
                                    3. Nakatani T et al: Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 45(4):1183-6, 2015
                                    4. Rosenbaum JN et al: Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 46(1):137-46, 2015
                                    5. Watanabe S et al: Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig. 53(4):149-55, 2015
                                    6. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                    7. Kusagaya H et al: Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 12:72, 2012
                                    8. Reddy TL et al: Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 40(2):377-85, 2012
                                    9. Piciucchi S et al: High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 12:111, 2011
                                    10. Frankel SK et al: Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 126(6):2007-13, 2004
                                    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

                                                • Pleuroparenchymal fibroelastosis (PPFE)
                                              • Synonyms

                                                • Amitani disease
                                              • Definitions

                                                • Rare interstitial pneumonia characterized by upper lobe-predominant elastotic fibrosis involving pleura and subpleural lung parenchyma
                                                  • Idiopathic or secondary (e.g., late complication of lung or hematopoietic stem cell transplantation)

                                              IMAGING

                                              • Radiographic Findings

                                                • CT Findings

                                                  • Nuclear Medicine Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Mariani F et al: Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagn Interv Radiol. 22(5):400-6, 2016
                                                                      2. Johkoh T et al: Rare idiopathic intestinal pneumonias (IIPs) and histologic patterns in new ATS/ERS multidisciplinary classification of the IIPs. Eur J Radiol. 84(3):542-6, 2015
                                                                      3. Nakatani T et al: Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 45(4):1183-6, 2015
                                                                      4. Rosenbaum JN et al: Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 46(1):137-46, 2015
                                                                      5. Watanabe S et al: Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig. 53(4):149-55, 2015
                                                                      6. Travis WD et al: An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 188(6):733-48, 2013
                                                                      7. Kusagaya H et al: Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 12:72, 2012
                                                                      8. Reddy TL et al: Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 40(2):377-85, 2012
                                                                      9. Piciucchi S et al: High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 12:111, 2011
                                                                      10. Frankel SK et al: Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 126(6):2007-13, 2004