Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.

Keywords: Niemann-Pick diseases; Tomography, X-ray computed; Lung diseases.

Objective: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. Methods: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. Results: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate. Conclusions: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

Keywords: Behcet Syndrome; Lung diseases, interstitial; Pulmonary circulation; Hypertension, pulmonary; Pulmonary embolism; Alveolar.

Objective: To quantify elastic fibers (EFs) and smooth muscle (SM) cells, as well as CD4+ and CD8+ T lymphocytes, in stable chronic obstructive pulmonary disease (COPD). Methods: Surgical specimens were obtained from 15 COPD patients, 18 smokers without airflow limitation, and 14 nonsmokers. Histological and immunohistochemical methods were employed in order to quantify EFs, SM cells, CD4+ T cells, and CD8+ T cells. Results: There was no significant difference in EF numbers among the three groups (p > 0.05). The number of EFs per unit area of lung tissue (mm2) and the percentage of EFs in the lung tissue were similar among the three groups. The numbers of SM cells were found to be higher in the COPD patients than in the smokers (p = 0.003) or in the nonsmokers (p = 0.009). There was a tendency toward an increase in CD8+ T-cell counts in the COPD patients. In specimens collected from the COPD patients, CD4+ T-cell counts were lower than in those collected from the smokers (p = 0.015) or from the nonsmokers (p = 0.003). There was a weak correlation between CD4+ T-cell count and the ratio of forced expiratory volume in one second to forced vital capacity (r2 = 0.003). Conclusions: The EF counts were similar among the three groups. Hypertrophy/hyperplasia of airway wall SM cells was found in the COPD patients and in the smokers, indicating that airway remodeling occurs in smokers. The CD4/CD8 ratio was lower in the COPD patients.

Keywords: Pulmonary disease, chronic obstructive; CD4 Lymphocyte Count; Elastic tissue; Muscle, smooth.

Background: Pulmonary involvement is common in leptospirosis and usually characterized by hemoptysis, dyspnea and diffuse bilateral infiltrates in chest X-rays. Such findings may be compatible with alveolar hemorrhage, already described by some authors both in autopsies and bronchoalveolar lavage (BAL). Objective: To evaluate the presence of alveolar hemorrhage, diagnosed through BAL, in bearers of leptospirosis patients with pulmonary involvement emphasizing the method's importance for early detection of this complication. Method: Seven patients with leptospirosis were submitted to BAL. All presented respiratory symptoms and/or infiltrates in the chest X-rays and/or hypoxemia. Alveolar hemorrhage was defined by the following findings in BAL: percentage of siderophages e"20% and/or Golde score >100 and/or hemorrhagic fluid. Culture and direct tests for leptospirosis were performed in BAL. Diagnosis of disease was confirmed by microscopy serum agglutination. Results: The aspect of the bronchoscopy was normal in five patients, showed blood in the bronchial tree in one case and inflammatory manifestations in another. The BAL aspect was hemorrhagic for all patients portraying alveolar hemorrhage. Culture and direct tests were negative for Leptospiras in the BAL. Conclusions: Leptospirosis must be taken into account in the differential diagnosis of alveolar hemorrhage. The BAL was confirmed as an efficient method for detection of alveolar hemorrhage in leptospirosis, to recommend immediate therapy for the purpose of preventing its evolution to massive hemoptysis and respiratory failure.

Keywords: Leptospirosis. Bronchoalveolar lavage fluid. Radiography, thoracic.

Objective: To evaluate HRCT scans of the chest in 22 patients with cocaine-induced pulmonary disease. Methods: We included patients between 19 and 52 years of age. The HRCT scans were evaluated by two radiologists independently, discordant results being resolved by consensus. The inclusion criterion was an HRCT scan showing abnormalities that were temporally related to cocaine use, with no other apparent causal factors. Results: In 8 patients (36.4%), the clinical and tomographic findings were consistent with "crack lung", those cases being studied separately. The major HRCT findings in that subgroup of patients included ground-glass opacities, in 100% of the cases; consolidations, in 50%; and the halo sign, in 25%. In 12.5% of the cases, smooth septal thickening, paraseptal emphysema, centrilobular nodules, and the tree-in-bud pattern were identified. Among the remaining 14 patients (63.6%), barotrauma was identified in 3 cases, presenting as pneumomediastinum, pneumothorax, and hemopneumothorax, respectively. Talcosis, characterized as perihilar conglomerate masses, architectural distortion, and emphysema, was diagnosed in 3 patients. Other patterns were found less frequently: organizing pneumonia and bullous emphysema, in 2 patients each; and pulmonary infarction, septic embolism, eosinophilic pneumonia, and cardiogenic pulmonary edema, in 1 patient each. Conclusions: Pulmonary changes induced by cocaine use are varied and nonspecific. The diagnostic suspicion of cocaine-induced pulmonary disease depends, in most of the cases, on a careful drawing of correlations between clinical and radiological findings.

Keywords: Cocaine, Cocaine-related disorders; Tomography, X-ray computed; Lung diseases.

Objective: To translate, adapt and validate the Patient Generated Index (PGI) for Brazilians with chronic obstructive pulmonary disease (COPD). Methods: 50 volunteers with COPD, mostly men (74%), with 73.1 ± 8.9 years of age, FEV1 of 52.3 ± 14.5% of predicted and FEV1 / FVC of 56.2 ± 8.6% of predicted responded to PGI, to the Saint George Respiratory Questionnaire (SGRQ) and to perform Glittre Activities of Daily Living test (Glittre ADL). After 1-2 weeks, PGI was again applied for the analysis of relative and absolute reliability. Results: The translation occurred without changes in the questionnaire. The score obtained in PGI had weak correlation with the SGRQ total score (r = -0.44, p <0.001) and with the impact domain (r = -0.40, p <0.05), presented a moderate correlation with the symptoms domain of the SGRQ (r = -0.55, p <0.001) and weak correlation with the activity domain (r = -0.31, p <0.05). A weak correlation was observed between PGI and Glittre ADL (r = -0.30; p <0.05). It was observed high reliability among the measures of PGI (ICCr = 0.94). Conclusion: This study shows that the Brazilian version of PGI is a reliable and valid instrument to measure health‑related quality of life (HRQL) in patients with COPD. It is a new and individualized form of evaluation of COPD patient-centered quality of life.

Keywords: Quality of life; Chronic obstructive pulmonary disease; Reproducibility of results and translations.

Objective: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). Methods: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. Results: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 ± 43 vs. 500.5 ± 90.3 IU/L); alanine aminotransferase (78.75 ± 37.7 vs. 162.75 ± 35.4 IU/L); alkaline phosphatase (160 ± 20.45 vs. 373.25 ± 45.44 IU/L); arterial oxygen tension (85.25 ± 8.1 vs. 49.9 ± 22.5 mmHg); and oxygen saturation (95 ± 0.7 vs. 73.3 ± 12.07%). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 ± 0.3 vs. 2.01 ± 0.9 nmol/mg protein); chemiluminescence (16008.41 ± 1171.45 vs. 20250.36 ± 827.82 cps/mg protein); and SOD (6.66 ± 1.34 vs. 16.06 ± 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. Conclusions: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.

Keywords: Hepatopulmonary syndrome; Lung; Oxidative stress; Rats.

Objective: This study sought to estimate body composition values by disease severity in patients recovered from COVID-19. Methods: This was an observational, analytical, prospective cross-sectional study involving patients recovered from COVID-19 and employing the following assessment methods: bioelectrical impedance analysis, dual-energy X-ray absorptiometry, and air displacement plethysmography. Results: A total of 210 volunteers were included. Demographic characteristics, comorbidities, and body composition values highlighted significant differences between men and women across disease severity levels. Conclusions: Sex differences influence the severity of COVID-19. Our results provide a cross-sectional analysis of the impact of body composition on COVID-19 patients, showing risk factors and parameters that can contribute to the treatment, health recovery, and quality of life of this population.

Keywords: COVID-19; Body composition; Risk factors; Quality of life.

Objective: To determine whether simple diagnostic methods can yield relevant disease information in patients with rheumatoid arthritis (RA). Methods: Patients with RA were randomly selected for inclusion in a cross-sectional study involving clinical evaluation of pulmonary function, including pulse oximetry (determination of SpO2, at rest), chest X-ray, and spirometry. Results: A total of 246 RA patients underwent complete assessments. Half of the patients in our sample reported a history of smoking. Spirometry was abnormal in 30% of the patients; the chest X-ray was abnormal in 45%; and the SpO2 was abnormal in 13%. Normal chest X-ray, spirometry, and SpO2 were observed simultaneously in only 41% of the RA patients. A history of smoking was associated with abnormal spirometry findings, including evidence of obstructive or restrictive lung disease, and with abnormal chest X-ray findings, as well as with an interstitial pattern on the chest X-ray. Comparing the patients in whom all test results were normal (n = 101) with those in whom abnormal test results were obtained (n = 145), we found a statistically significant difference between the two groups, in terms of age and smoking status. Notably, there were signs of airway disease in nearly half of the patients with minimal or no history of tobacco smoke exposure. Conclusions: Pulmonary involvement in RA can be identified through the use of a combination of diagnostic methods that are simple, safe, and inexpensive. Our results lead us to suggest that RA patients with signs of lung involvement should be screened for lung abnormalities, even if presenting with no respiratory symptoms.

Keywords: Arthritis, rheumatoid; Lung diseases, interstitial; Spirometry; Radiography, thoracic; Airway ob-struction.

Objective: To assess the risk factors for interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) and to evaluate the association of ILD with the use of methotrexate as well as with joint disease activity. Methods: A retrospective, cross-sectional study conducted between March and December 2019 at a tertiary healthcare center, in a follow-up of RA patients who had undergone pulmonary function tests (PFT) and chest computed tomography. We evaluated the tomographic characteristics, such as the presence of ILD and its extension, as well as joint disease activity. Functional measurements, such as forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), were also assessed. After this, a multivariate logistic regression analysis was applied in order to identify risk factors associated with ILD. Results: We evaluated 1.233 patients, of which 134 were eligible for this study. The majority were female (89.6%), with a mean age of 61 years old and with a positive rheumatoid factor (86.2%). RA-associated ILD (RA-ILD) was detected in 49 patients (36.6%). We found an association of RA-ILD with age == 62 year, male sex, smoking history and fine crackles in lung auscultation and a decreased DLCO. The indicators of being aged = 62 years old and having moderate or high RA disease activity were both independent factors associated with RA-ILD, with an odds ratio of 4.36 and 3.03, respectively. The use of methotrexate was not associated with a higher prevalence of ILD. Conclusion: Age and RA disease activity are important risk factors associated with RA-ILD. Methotrexate was not associated with the development of RA-ILD in the present study.

Keywords: Rheumatoid arthritis; Interstitial lung disease; Rheumatoid arthritis- associated interstitial lung disease.

Objective: To evaluate the effects that pulmonary resection has on pulmonary function and quality of life (QoL) in patients with primary or metastatic lung cancer. Methods: This was a prospective cohort study involving all patients submitted to pulmonary resection for cancer between September of 2006 and March of 2007 at the A. C. Camargo Hospital in São Paulo, Brazil. Patients underwent spirometry in the preoperative period and at six months after the surgical procedure. After a postoperative period of six months, the patients completed an overall QoL questionnaire (the Medical Outcomes Study 36-item Short-form Health Survey) and another one, specific for respiratory symptoms (the Saint George's Respiratory Questionnaire). The scores obtained in our study were compared with those previously obtained for a general population and for a population of patients with COPD. Results: We included 33 patients (14 males and 19 females), ranging in age from 39 to 79 years. All of the patients, smokers and nonsmokers alike, presented significant worsening of pulmonary function. The mean scores on the overall QoL questionnaire were approximately 5% lower than those obtained for the general population. The scores of various domains of the symptom-specific QoL questionnaire were 50-60% lower than those obtained for the general population and approximately 20% higher than those obtained for the population with COPD. Conclusions: Pulmonary resection has a direct negative impact on pulmonary function and QoL, especially on the QoL related to aspects directly linked to pulmonary function. We highlight the importance of preoperative assessment of pulmonary function in patients undergoing pulmonary resection, in order to predict their postoperative evolution.

Keywords: Thoracic surgery; Lung neoplasms/surgery; Spirometry; Quality of life; Questionnaires.