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
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
Related Anatomy
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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
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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