Combined pulmonary fibrosis and emphysema syndrome: a radiologic perspective.

Chronic obstructive pulmonary disease (that includes emphysema) results in significant morbidity and mortality worldwide. Idiopathic pulmonary fibrosis (IPF) is also a chronic and progressive parenchymal lung disease with an average survival of less than 5 years after diagnosis. Combined pulmonary fibrosis and emphysema (CPFE) is an important but still underdiagnosed syndrome. Its diagnosis is based on the radiological findings at computed tomography which consists of emphysema of the upper lung zones and fibrosis of the lower lung zones. Since this syndrome has a very bad prognosis, even worse than isolated finding of emphysema or fibrosis alone, early recognition and rapid treatment are important. In this article we will review and elucidate the radiologic appearance of this syndrome and highlight its clinical importance.

Comprehensive exercise training improves ventilatory muscle function and reduces dyspnea perception in patients with COPD.

BACKGROUND: Comprehensive exercise training (CET) is an efficient strategy to decrease dyspnea perception in chronic obstructive pulmonary disease (COPD) and may result in significant improvement in ventilatory muscles function. Our aim was to evaluate the effects of general exercise training on dyspnea perception and on respiratory muscles strength in COPD patients. METHODS: Consecutive COPD patients were enrolled to complete a CET programme. The patients underwent a routine that included a global warm up, upper and lower limbs endurance exercise as well as stretching and relaxation. Before and after the CET programme, patients completed maximal inspiratory (PImax) and expiratory (PEmax) pressures measurements, maximal incremental test, endurance test, and 6-min walk distance (6MWD). RESULTS: 71 patients (52 male). Mean age 67.6 +/- 8.6 years, FEV1 (%) 44.2 +/- 16.2 and Mahler dyspnea scale 6.4 +/- 1.8. The results before and after the exercise programme were: PImax 64.7 +/- 22.9 vs. 75.5 +/- 23.7 cmH2O (p=0.001), PEmax 110.8 +/- 28.1 vs. 120.4 +/- 28.1 cmH2O (p=0.004), 6MWD 510.6 +/- 90.3 vs. 528.2 +/- 99.7 metres (p=0.88), time of incremental test 672 +/- 135 vs. 856 +/- 226 sec (p<0.0001). Compared with the pre exercise programme, we observed a significant reduction on Borg dyspnea scale (6.1 +/- 2.8 to 3.6 +/- 2.3, p<0.0001) as well as a longer test time (504 +/- 218 to 1.038 +/- 841, p<0.0001) at the end of the endurance test after CET programme. Improvement of PImax correlated negatively with dyspnea perception at iso-time during the endurance test (r = -0.33, p=0.03). CONCLUSIONS: Our results confirm that CET is associated with significant improvement in PImax, PEmax and provide evidence demonstrating that CET reduces dyspnea perception in patients with COPD.

Effects of hyperinflation on the oxygen pulse as a marker of cardiac performance in COPD.

A decreased inspiratory capacity (IC)/total lung capacity (TLC) ratio is associated with dynamic hyperinflation and decreased exercise capacity. The present authors hypothesised that static (low IC/TLC) and dynamic hyperinflation impair cardiac function as assessed by oxygen pulse at rest and during cardiopulmonary exercise testing (CPET). Lung function, body mass index, hand grip strength and CPET parameters were measured (oxygen uptake (mL x kg(-1) x min(-1)) and oxygen pulse (mL x beat(-1))) in 87 chronic obstructive pulmonary disease (COPD) patients (American Thoracic Society/European Respiratory Society/Global Initiative for Chronic Obstructive Lung Disease stage 3-4) and 46 controls. The patients were divided into those with IC/TLC > 25% or < or = 25%. The IC/TLC ratio at rest and at peak exercise was associated significantly with oxygen pulse. Patients with IC/TLC < or = 25% (n = 45) had significantly lower exercise capacity, peak oxygen pulse, peak minus baseline oxygen pulse, peak IC, peak IC/TLC ratio and % change from baseline to peak IC/TLC ratio compared with patients with IC/TLC > 25% and controls. During CPET, the oxygen pulse was lower at iso-work in patients with IC/TLC < or = 25% than in those with IC/TLC > 25%. Resting hyperinflation (inspiratory capacity/total lung capacity) is associated with lower oxygen pulse, peak exercise inspiratory capacity/total lung capacity and exercise capacity in patients with severe chronic obstructive pulmonary disease. The present results support an interaction between hyperinflation and decreased cardiac function that may contribute to exercise limitation in these patients.

Effect of sitaxsentan treatment on quality of life in pulmonary arterial hypertension.

In recent years new therapeutic options have been developed for the management of pulmonary arterial hypertension (PAH). Sitaxsentan is an oral, once daily, highly selective endothelin A receptor antagonist that recently demonstrated a positive effect on functional capacity and haemodynamics of PAH patients. The aim of this study is to evaluate the effect of sitaxsentan in the quality of life (QOL) of PAH patients. Twenty-three patients with idiopathic PAH or PAH associated to collagen vascular diseases were evaluated at baseline and after 16 weeks of treatment with sitaxsentan 100 mg orally, once daily. 6-min walk test distance (6MWD) and QOL questionnaire (QOLQ) (SF-36) were obtained at baseline and at week 16. There was a significant improvement in functional capacity evaluated by 6MWD (472 m vs. 490 m, p = 0.03) and also in the physical component of the QOLQ (p < 0.01). Evaluating each of the domains of the SF-36 QOLQ, those more related to physical capacity presented a significant increase while the domains related to the mental component presented a trend of improvement, without reaching statistical significance. Sitaxsentan improves QOL in patients with PAH mainly through the domains related to functional capacity.