The Role of Computed Tomography for the Evaluation of Lung Disease in Alpha-1 Antitrypsin Deficiency
| Autor: | Michael Campos, Alejandro A. Diaz |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Lung Diseases
A1-PI alpha-1 proteinase inhibitor Pulmonary and Respiratory Medicine PD15 15th percentile density Pathology medicine.medical_specialty VI voxel index Critical Care and Intensive Care Medicine Pulmonary function testing FRC functional residual capacity 03 medical and health sciences 0302 clinical medicine Functional residual capacity alpha 1-Antitrypsin Deficiency Parenchyma Humans COPD AATD alpha-1 antitrypsin deficiency Medicine Lung volumes 030212 general & internal medicine 3-D three-dimensional alpha-1 antitrypsin deficiency 2-D two-dimensional Lung Alpha 1-antitrypsin deficiency biology business.industry alpha-1 proteinase inhibitor TLC total lung capacity respiratory system Recent Advances in Chest Medicine medicine.disease respiratory tract diseases emphysema medicine.anatomical_structure 030228 respiratory system HU Hounsfield unit Neutrophil elastase biology.protein alpha-1 antitrypsin Tomography X-Ray Computed Cardiology and Cardiovascular Medicine business CT |
| Zdroj: | Chest |
| ISSN: | 0012-3692 |
| DOI: | 10.1016/j.chest.2017.11.017 |
| Popis: | Alpha-1 antitrypsin deficiency (AATD) is characterized by low serum levels of or dysfunctional alpha-1 proteinase inhibitor. In the lung parenchyma, this results in a loss of protection against the activity of serine proteases, particularly neutrophil elastase. The resultant imbalance in protease and antiprotease activity leads to an increased risk for the development of early-onset emphysema and COPD. As in traditional smoke-related COPD, the assessment of the severity and disease progression of lung disease in AATD is conventionally based on lung function; however, pulmonary function tests are unable to discriminate between emphysema and airways disease, the two hallmark pathologic features of COPD. CT imaging has been used as a tool to further characterize lung structure and evaluate therapeutic interventions in AATD-related COPD. Moreover, recent advances in quantitative CT have significantly improved our assessment of the lung architecture, which has provided investigators and clinicians with a more detailed evaluation of the extent and severity of emphysema and airways disease in AATD. In addition, serial CT imaging measures are becoming increasingly important, as they provide a tool to monitor emphysema progression. This review describes the principles of CT technology and the role of CT imaging in assessing pulmonary disease progression in AATD, including the effect of therapeutic interventions. |
| Databáze: | OpenAIRE |
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