Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

Keywords: Pulmonary fibrosis; Emphysema; Hypertension, pulmonary; Lung diseases, interstitial.

Objective: To describe the clinical and functional findings recently reported in the medical literature for patients diagnosed with emphysema involving the upper lobes and idiopathic pulmonary fibrosis (IPF) involving the lower lobes. Methods: Eleven patients with emphysema and IPF were identified retrospectively. All of the patients underwent high-resolution computed tomography of the lung and pulmonary function tests. Results: Of the 11 patients, 8 were male and 3 were female. The mean age was 70.7 ± 7.2 years (range, 61-86 years). All of the patients were smokers (mean smoking history, 61.5 ± 43.5 pack-years). The mean values of forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and FEV1/FVC were 72.1 ± 12.7%, 68.2 ± 11.9% and 74.4 ± 10.8, respectively. Lung volumes were normal in 7 patients. A restrictive pattern was observed in 3 patients, and hyperinflation was present in one. The diffusing capacity was moderatelyto- severely reduced in all of the patients (mean, 27.7% ± 12.9% of predicted). Ten of the 11 patients performed the six-minute walk test. The mean distance covered was 358.4 ± 143.1 m, and 9 of the 10 patients presented desaturation ≥ 4%. Echocardiographic findings suggestive of pulmonary hypertension were present in 4 patients (mean systolic pulmonary artery pressure, 61.8 mmHg; range, 36-84 mmHg). Conclusions: The concomitant presence of emphysema and IPF causes characteristic changes on pulmonary function tests. The most significant finding is a discrepancy between diffusing capacity and spirometry results.

Keywords: Pulmonary emphysema; Pulmonary fibrosis; Lung Diseases, interstitial; Anoxia; Hypertension, pulmonary.

Objective: The rapid differentiation between Mycobacterium tuberculosis and nontuberculous mycobacteria is fundamental for patients co-infected with tuberculosis and HIV. To that end, we use two methods in our laboratory: detection of cord factor and PCR-restriction enzyme analysis (PRA). The objective of this study was to evaluate the accuracy of a screening test on solid medium as a rapid method for the presumptive identification of M. tuberculosis complex, considering costs and turnover time. Methods: A total of 152 strains were submitted to a combined screening test, consisting of the detection of cord factor under microscopy (Ziehl-Neelsen staining) and evaluation of the macroscopic aspect of colonies, as well as to PRA, which was used as the gold standard. Costs were estimated by calculating the price of all of the materials needed for each test. Results: The overall accuracy of cord factor detection alone was 95.4% (95% CI: 90.7-98.1%), and that of the combined screening test was 99.3% (95% CI: 96.4-100%). Cord factor detection costs US$ 0.25, whereas the PRA costs US$ 7.00. Results from cord factor detection are ready in 2 days, whereas PRA requires 4 days to yield results. Conclusions: The presumptive identification of M. tuberculosis using the macroscopic evaluation of colonies combined with cord factor detection under microscopy is a simple, rapid and inexpensive test. We recommend the combined screening test to rapidly identify M. tuberculosis in resource-poor settings and in less well-equipped laboratories while awaiting a definite identification by molecular or biochemical methods.

Keywords: Tuberculosis; Mycobacterium/classification; Polymerase chain reaction; Diagnostic tests, routine.

Emphysema is a condition of the lung, characterized by the abnormal increase in the size of the airspace distal to the terminal bronchioles. Currently, emphysema is the fourth leading cause of death in the USA, affecting 14 million people. The present article describes the principal tools in the imaging diagnosis of emphysema, from the early days until the present. We describe traditional techniques, such as chest X-ray, together with the evolution of computed tomography (CT) to more advanced forms, such as high resolution CT, as well as three-dimensional CT densitometry and volumetric assessment.

Keywords: Emphysema; Radiology; Tomography, X-Ray computed; Radiography, thoracic.

Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.

Keywords: Idiopathic pulmonary fibrosis/diagnosis; Idiopathic pulmonary fibrosis/physiopathology; Idiopathic pulmonary fibrosis/therapy; Idiopathic pulmonary fibrosis/rehabilitation.

Objective: The reproduction of an experimental emphysema model in rats by intratracheal instillation of papain was proposed for the evaluation of lung volume reduction surgeries. Method: The study used orotracheal instillation of papain (20 mg/kg) dissolved in 0.9% saline solution. Forty days after instillation, animals underwent mechanical ventilation. Elastance and resistance of the respiratory system were measured, and the rats were sacrificed by having their lungs removed. The pulmonary tissue of the animals was qualitatively analyzed with hematoxylineosin staining and submitted to morphometric analysis for the measurement of the mean alveolar diameter. The pulmonary tissue was also subjected to resorcin-fuchsin staining for the identification of elastic fibers, which were quantified in alveolar septa by digital imaging. Results: In the animals instilled with papain, the histological analysis of the lungs showed pan-acinar emphysema, with rupture of alveolar septa and hyperdistention. Morphometric analysis showed higher mean values for mean alveolar diameter in the lungs of the animals submitted to papain (149.08 μm and 100.56 μm) as compared to the group receiving saline solution (64.08 μm and 75.90 μm). The quantification of elastic fibers of alveolar septa of papain-treated animals was 70% lower than in the animals receiving saline solution. Mechanic ventilation did not show differences in respiratory system resistance of animals receiving papain or saline solution. Respiratory system elastance was lower in the group receiving papain than in the group receiving saline solution, showing a functional status compatible with pulmonary emphysema, with decreased elasticity of the pulmonary tissue. Conclusion: The reproduction of an experimental model of pan-acinar pulmonary emphysema in rats was achieved by papain instillation through the respiratory tree with functional and morphologic evidences.

Keywords: Pulmonary emphysema. Experimental model. Papain. Rats. Drug instillations.

Congenital lobar emphysema is a disease in which symptoms often begin during the first six months of age. In this report, the authors present the case of a twenty-year-old woman whose symptoms started when she had a spontaneous pneumothorax, an exceptional occurrence. The possible etiologies for that congenital lung malformation and the reasons for the performance of a conservative treatment are discussed.

Keywords: Pulmonary emphysema. Pneumothorax. Abnormalities.

Objective: To present a locally developed system of computer vision for use with HRCT images, designated SIStema para a Detecção e a quantificação de Enfisema Pulmonar (SISDEP, System to Detect and Quantify Pulmonary Emphysema), and to compare this system with a freeware system tool. Methods: Thirty-three HRCT images scanned at the apex, hilum and base of the lungs of 11 patients with COPD were analyzed. The SISDEP was compared with the Osiris Medical Imaging Software Program regarding lung parenchyma segmentation, precision of the measurement of the cross-sectional area of the lungs in mm2, mean lung density (MLD), relative area (RA) of the lung occupied by voxels with attenuation values < −950 Hounsfield units (RA −950), 15th percentile point (Perc15) and visualization of hyperinflated areas using a color mask. Results: Although both computational systems were efficient in segmenting the lungs, the SISDEP performed this task automatically and more rapidly. There were significant correlations between the two systems in terms of the results obtained for lung cross-sectional area, MLD, RA −950 and Perc15 (r2 = 0.99, 0.99, 0.99 and 1.00, respectively). The color mask tool of the SISDEP allowed excellent visualization of hyperinflated areas, discriminating them from normal areas. Conclusions: The SISDEP was efficient in segmenting the lungs and quantifying lung hyperinflation, presenting an excellent correlation with the Osiris system. The SISDEP constitutes a promising computational tool for diagnosing and assessing the progression of emphysema in HRCT images of COPD patients.

Keywords: Pulmonary disease, chronic obstructive; Emphysema; Tomography, X-ray computed; Decision making, computer-assisted.

Spontaneous pneumomediastinum is an uncommon event, the clinical picture of which includes retrosternal chest pain, subcutaneous emphysema, dyspnea, and dysphonia. The pathophysiological mechanism involved is the emergence of a pressure gradient between the alveoli and surrounding structures, causing alveolar rupture with subsequent dissection of the peribronchovascular sheath and infiltration of the mediastinum and subcutaneous tissue with air. Known triggers include acute exacerbations of asthma and situations that require the Valsalva maneuver. We described and documented with HRCT scans the occurrence of pneumomediastinum after a patient with bleomycin-induced interstitial lung disease underwent pulmonary function testing. Although uncommon, the association between pulmonary function testing and air leak syndromes has been increasingly reported in the literature, and lung diseases, such as interstitial lung diseases, include structural changes that facilitate the occurrence of this complication.

Keywords: Mediastinal emphysema; Subcutaneous emphysema; Spirometry; Bleomycin.

Background: Idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. Consequently, the prognostic value of histological findings needs to be reassessed. Objective: To correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. Method: Patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males) were evaluated. Of the 51, 26 were smokers or ex-smokers. Duration of symptoms, forced vital capacity and smoking habits were recorded. All patients presented usual interstitial pneumonia verified through histology. Degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. Results: Median duration of symptoms was 12 months and initial forced vital capacity was 72 ± 21%. Cox multivariate analysis revealed that survival correlated inversely and significantly (p < 0.05) with duration of symptoms and fibroblastic foci score, as well as with myointimal thickening of blood vessels. Limited numbers of fibroblastic foci, as well as myointimal thickening involving less than 50% of blood vessels, were predictive of greater survival. No correlation with survival was found for gender, age, forced vital capacity, inflammation or degree of cellularity. Conclusion: Semiquantitative analysis of lung biopsies yields relevant prognostic information regarding patients with usual interstitial pneumonia.

Keywords: Pulmonary fibrosis. Lung diseases, interstitial. Survival analysis.

Over the last five years, knowledge in the field of pulmonary hypertension has grown consistently and significantly. On the basis of various clinical studies showing the usefulness of new diagnostic tools, as well as the efficacy of new medications and drug combinations, new diagnostic and treatment algorithms have been developed. Likewise, in order to simplify the clinical management of patients, the classification of pulmonary hypertension has been changed in an attempt to group the various forms of pulmonary hypertension in which the diagnostic and therapeutic approaches are similar. The objective of this review was to discuss these modifications, based on the 2005 Brazilian guidelines for the management of pulmonary hypertension, emphasizing what has been added to the international guidelines.

Keywords: Hypertension, pulmonary/diagnosis; Hypertension, pulmonary/therapy; Clinical protocols.