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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)
- Rare interstitial pneumonia characterized by upper lobe-predominant elastotic fibrosis involving pleura and subpleural lung parenchyma
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
- 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
- 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
- Nakatani T et al: Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 45(4):1183-6, 2015
- Rosenbaum JN et al: Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 46(1):137-46, 2015
- Watanabe S et al: Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig. 53(4):149-55, 2015
- 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
- Kusagaya H et al: Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 12:72, 2012
- Reddy TL et al: Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 40(2):377-85, 2012
- Piciucchi S et al: High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 12:111, 2011
- Frankel SK et al: Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 126(6):2007-13, 2004
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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)
- Rare interstitial pneumonia characterized by upper lobe-predominant elastotic fibrosis involving pleura and subpleural lung parenchyma
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
- 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
- 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
- Nakatani T et al: Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 45(4):1183-6, 2015
- Rosenbaum JN et al: Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 46(1):137-46, 2015
- Watanabe S et al: Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig. 53(4):149-55, 2015
- 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
- Kusagaya H et al: Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 12:72, 2012
- Reddy TL et al: Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 40(2):377-85, 2012
- Piciucchi S et al: High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 12:111, 2011
- Frankel SK et al: Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 126(6):2007-13, 2004
