Quantitative assessment of emphysema distribution in smokers and patients with α1-antitrypsin deficiency

SummaryIntroductionIdentification of upper lobe emphysema is mandatory before lung volume reduction surgery (LVRS). Here we introduce a CT-based objective model for describing the distribution of different types of emphysema.MethodsFifty COPD patients were included in the study. Half had α1-antitrypsin deficiency (α1-COPD) and the rest had smoking-induced emphysema (usual COPD). All patients were scanned 3 times. The relative area of emphysema in each CT slice was plotted against table position, and the cranio-caudal distribution was calculated as the slope of the regression line.ResultsThe variation in slopes within a patient was much less than the variation in slopes between patients (P<0.0001). There was a significant difference between slopes in the α1-COPD and the usual COPD groups (P<0.0001). In the α1-COPD group, 24/25 patients had lower lobe emphysema. In the usual COPD group, 4 patients had upper lope predominance, 5 patients had heterogeneous distributions, and 16 patients had lower lobe predominance.ConclusionsThe majority of patients with smoking-related emphysema have a homogeneous distribution and lower lobe predominance although not as noticeable as in α1-antitrypsin deficiency. An objective and quantitative method for determining the distribution of emphysema should be applied when selecting candidates for LVRS

Idiopathic pulmonary fibrosis: Best practice in monitoring and managing a relentless fibrotic disease

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease that is, by definition, progressive. Progression of IPF is reflected by a decline in lung function, worsening of dyspnea and exercise capacity, and deterioration in health-related quality of life. In the short term, the course of disease for an individual patient is impossible to predict. A period of relative stability in forced vital capacity (FVC) does not mean that FVC will remain stable in the near future. Frequent monitoring using multiple assessments, not limited to pulmonary function tests, is important to evaluate disease progression in individual patients and ensure that patients are offered appropriate care. Optimal management of IPF requires a multidimensional approach, including both pharmacological therapy to slow decline in lung function and supportive care to preserve patients' quality of life