Autosomal recessive disorder; mutations of CF transmembrane conductance regulator (CFTR) gene; regulates chloride transport
Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
Multiorgan involvement; primarily lungs and pancreas
Most common fatal hereditary disease in Caucasians
Accounts for up to 25% of adult cases of bronchiectasis
Variable severity attributed to spectrum of CFTR mutations
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
McCallister A et al: Comparison of single breath hyperpolarized 129 Xe MRI with dynamic 19 F MRI in cystic fibrosis lung disease. Magn Reson Med. 85(2):1028-38, 2021
Almughem FA et al: Cystic fibrosis: overview of the current development trends and innovative therapeutic strategies. Pharmaceutics. 12(7), 2020
Benlala I et al: Quantification of MRI T2-weighted high signal volume in cystic fibrosis: a pilot study. Radiology. 294(1):186-96, 2020
Chassagnon G et al: Quantification of cystic fibrosis lung disease with radiomics-based CT scores. Radiol Cardiothorac Imaging. 2(6):e200022, 2020
Lattanzi C et al: Allergic bronchopulmonary aspergillosis in children with cystic fibrosis: an update on the newest diagnostic tools and therapeutic approaches. Pathogens. 9(9), 2020
Renner S et al: Update on respiratory fungal infections in cystic fibrosis lung disease and after lung transplantation. J Fungi (Basel). 6(4), 2020
Revel MP et al: Use of MRI to measure bronchial inflammation in cystic fibrosis. Radiology. 294(1):197-8, 2020
Bergeron C et al: Cystic fibrosis: pathophysiology of lung disease. Semin Respir Crit Care Med. 40(6):715-26, 2019
Burke AP et al: Bronchiectasis. In Practical Thoracic Pathology: Diseases of the Lung, Heart, and Thymus. Wolters Kluwer.137-41, 2017
Nyilas S et al: Novel magnetic resonance technique for functional imaging of cystic fibrosis lung disease. Eur Respir J. 50(6), 2017
Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
Hasleton P et al: Congenital abnormalities and pediatric lung diseases, including neoplasms. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 97-100, 2013
Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
Cystic fibrosis (CF)
Synonyms
Mucoviscidosis
Definitions
Autosomal recessive disorder; mutations of CF transmembrane conductance regulator (CFTR) gene; regulates chloride transport
Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
Multiorgan involvement; primarily lungs and pancreas
Most common fatal hereditary disease in Caucasians
Accounts for up to 25% of adult cases of bronchiectasis
Variable severity attributed to spectrum of CFTR mutations
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
McCallister A et al: Comparison of single breath hyperpolarized 129 Xe MRI with dynamic 19 F MRI in cystic fibrosis lung disease. Magn Reson Med. 85(2):1028-38, 2021
Almughem FA et al: Cystic fibrosis: overview of the current development trends and innovative therapeutic strategies. Pharmaceutics. 12(7), 2020
Benlala I et al: Quantification of MRI T2-weighted high signal volume in cystic fibrosis: a pilot study. Radiology. 294(1):186-96, 2020
Chassagnon G et al: Quantification of cystic fibrosis lung disease with radiomics-based CT scores. Radiol Cardiothorac Imaging. 2(6):e200022, 2020
Lattanzi C et al: Allergic bronchopulmonary aspergillosis in children with cystic fibrosis: an update on the newest diagnostic tools and therapeutic approaches. Pathogens. 9(9), 2020
Renner S et al: Update on respiratory fungal infections in cystic fibrosis lung disease and after lung transplantation. J Fungi (Basel). 6(4), 2020
Revel MP et al: Use of MRI to measure bronchial inflammation in cystic fibrosis. Radiology. 294(1):197-8, 2020
Bergeron C et al: Cystic fibrosis: pathophysiology of lung disease. Semin Respir Crit Care Med. 40(6):715-26, 2019
Burke AP et al: Bronchiectasis. In Practical Thoracic Pathology: Diseases of the Lung, Heart, and Thymus. Wolters Kluwer.137-41, 2017
Nyilas S et al: Novel magnetic resonance technique for functional imaging of cystic fibrosis lung disease. Eur Respir J. 50(6), 2017
Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
Hasleton P et al: Congenital abnormalities and pediatric lung diseases, including neoplasms. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 97-100, 2013
Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005
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