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.

Behçet's disease is a systemic disease with clinical manifestations, whose underlying histopathologic lesion is a non-specific vasculitis. Pulmonary artery aneurysms and, more rarely, pleuropulmonary manifestations may be found. Some reports are available in the literature about the use of immunosuppressive agents in these conditions. The authors describe a case in whom clinical remission and radiologic resolution were observed after corticosteroid and cyclophophamide therapy.

Keywords: Behçet's disease. Pulmonary artery aneurysms.

The authors present a case of a patient with Behçet's disease who complained about sudden dyspnea while performing light physical exercises, about non-productive cough, weakness, and frontal headache; he also presented significant weight loss in the last month while being treated for an alleged tuberculosis. After going through an episode of massive hemophthisis, his clinical history and physical examination were reevaluated and his new diagnosis was concluded to be Behçet's disease. Thus, the authors started treatment by giving immunosuppressive drugs and corticosteroids and, as a consequence, regression of the aneurysm was noted. In this article the authors discuss the clinical, radiological, histopathologic and therapeutic features of this syndrome.

Keywords: Behçet's disease. Pulmonary arterial aneurysms.

Objective: To analyze the effects of the continuous positive airway pressure (CPAP), with 30% of FiO2 and in compressed air, used during the performance of submaximal physical activity on an ergometric treadmill. Materials and methods: Nine male individuals with COPD were submitted to physical activity on the ergometric treadmill using CPAP with 30% FiO2 .The authors evaluated the behavior of the spirometric parameters, the forced vital capacity (FVC), the forced expiratory volume in the first second (FEV1) before and after physical activity, the respiratory frequency (f), the arterial oxygen saturation (SaO2), the cardiac frequency (cf), the systolic arterial pressure (SAP), and the diastolic arterial pressure (DAP) at rest and during physical activity. Results and conclusion: Results have shown that, when using CPAP in both situations analyzed, there has been an increase in the FVC and a decrease in f and in cf with statistically significant difference (α = 0.05). SaO2 has showned a significantly higher mean when CPAP was used with 30% of FiO2 (α = 0.05). The FEV1, the SAP and DAP have maintained the same value in the statistical analyses.

Keywords: Exercise test. Forced expiratory volume. Vital capacity.

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 study the impact that heated humidification instituted in the beginning of automatic positive airway pressure (APAP) therapy has on compliance with and the side effects of the treatment. Methods: Thirty-nine treatment-naïve patients with obstructive sleep apnea were randomized into two groups to receive APAP using one of two modalities: with heated humidification (APAPwith group); and without heated humidification (APAPw/o group).Patients were evaluated at 7 and 30 days after APAP initiation. The following parameters were analyzed: compliance with treatment (mean number of hours/night); side effects (dry nose or mouth, nasal obstruction and rhinorrhea); daytime sleepiness (Epworth sleepiness scale score) and subjective comfort (visual analog scale score). Patients were also evaluated in terms of residual apnea-hypopnea index (AHI), as well as mean pressures and leaks registered in the ventilators. Results: There were no differences between the two groups in terms of mean age (APAPwith: 57.4 ± 9.2; APAPw/o: 56.5 ± 10.7 years), AHI (APAPwith: 28.1 ± 14.0; APAPw/o: 28.8 ± 20.5 events/hour of sleep), baseline Epworth score (APAPwith: 11.2 ± 5.8; APAPw/o: 11.9 ± 6.3) and initial nasal symptoms. Compliance was similar in both groups (APAPwith: 5.3 ± 2.4; APAPw/o: 5.2 ± 2.3 h/night). There were no differences in any of the other parameters analyzed. Conclusions: The introduction of heated humidification at the beginning of APAP therapy provided no advantage in terms of treatment compliance or side effects of treatment.

Keywords: Humidity; Positive-pressure respiration/adverse effects; Patient compliance.

Objective: Assess the relationship between adherence to long-term oxygen therapy (LTOT) with mortality in patients with chronic obstructive pulmonary disease (COPD) and chronic respiratory failure and their clinical features. Methods: Longitudinal retrospective analysis of 254 patients with COPD and chronic respiratory failure from 2008 to 2016. At baseline, we evaluated the diagnosis, spirometry values, arterial blood gas analysis, blood count, pulse oximetry, body composition and health questionnaires (dyspnea, quality of life, anxiety and depression). For referred adherence analysis to LTOT we included 199 patients, divided according to prescription of oxygen: 12h/day (G1), 15h/day (G2) and 24h/day (G3). The cause of death and dates were studied over the five-year period. Results: In five years we identified 124 deaths (62.3%). No significant difference was found in mortality between the adherence groups (p=0.75) nor did we find differences in the clinical parameters evaluated. LTOT prescription was not associated with mortality (p=0.07). In Cox regression analysis, there was no association between mortality and non-adherence to LTOT (HR: 0.75; IC95%: 0.21-2.70). The risk of mortality was increased in G3 compared with G1 (HR: 7.16; IC 95%: 1.44-35.38) and in those with a higher depression score (HR: 1.35; IC: 1.14-1.59). Conclusion: No association was found between LTOT adherence and mortality in patients with COPD and respiratory failure. There were no clinical differences between the adherence groups.

Keywords: Chronic obstructive pulmonary disease; Oxygen therapy; Cooperation and treatment adherence; Morbidity and mortality indicators.

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.

Abstract Objective: To determine the validity and reproducibility of two subjective instruments to assess limitations in activities of daily living (ADLs) in patients with chronic obstructive pulmonary disease (COPD) in Brazil: the Pulmonary Functional Status and Dyspnea ­Questionnaire ‑ ­Modified version (PFSDQ-M) and the Medical Research Council (MRC) scale. Methods: Thirty patients with COPD (age, 67 ± 10 years; males, 17; forced expiratory volume in one second, 42% ± 13% of predicted) completed the Portuguese-language versions of the two instruments on two occasions, one week apart. The PFSDQ-M has three components: influence of dyspnea on ADLs, influence of fatigue on ADLs and change experienced by the patient with ADLs. The MRC scale is simple, with only five items, in which patients report the degree to which dyspnea limits their performance of ADLs. The traditional Saint George's Respiratory Questionnaire (SGRQ), previously validated for use in Brazil, was used as a validation criterion. Results: The test-retest reliability (intraclass correlation coefficient) of the PFSDQ-M was 0.93, 0.92 and 0.90 for the dyspnea, fatigue and change components, respectively, compared with 0.83 for the MRC scale. Bland-Altman plots showed good test-retest concordance for the PFSDQ-M. The components of the PFSDQ-M and the MRC scale correlated significantly with all of the domains and the total score on the SGRQ (0.49 < r < 0.80; p < 0.01 for all). Conclusions: The Portuguese-language versions of the PFSDQ-M and the MRC scale proved reproducible and valid for use in patients with COPD in Brazil.

Keywords: Pulmonary disease, chronic obstructive; Activities of daily living; Validation studies.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which most patients die of respiratory failure. Although volume-targeted non-invasive bilevel positive airway pressure (BPAP) ventilation has been studied in patients with chronic respiratory failure of various etiologies, its use in ALS has not been reported. We present the case of a 66-year-old woman with ALS and respiratory failure treated with volume-targeted BPAP ventilation for 15 weeks. Weekly data downloads showed that disease progression was associated with increased respiratory muscle weakness, decreased spontaneous breathing, and increased use of non-invasive positive pressure ventilation, whereas tidal volume and minute ventilation remained relatively constant.

Keywords: Amyotrophic lateral sclerosis; Respiratory insufficiency; Hypoventilation; Intermittent positive-pressure ventilation; Sleep.

Objective: To translate the London Chest Activity of Daily Living (LCADL) scale into Portuguese and to determine whether this version is reproducible in Brazilian patients with severe chronic obstructive pulmonary disease (COPD). Methods: The LCADL scale was translated into Portuguese and then back-translated into English. This pilot Brazilian Portuguese version was administered to 8 patients with COPD, and possible text-related problems were investigated. The principal problems were discussed with the authors of the original scale, and a final translated version was arrived at. At the study outset, two observers administered this final version (twice in one day) to 31 patients with COPD. One of those observers again administered the scale to the same patients 15-20 days later. At baseline, the patients were submitted to pulmonary function testing and to the six-minute walk test (6MWT). Results: The Brazilian Portuguese version of the LCADL scale demonstrated excellent reproducibility in the total score and in most of the questions, with an inter-rater Cronbach's alpha coefficient of 0.97 (95% CI: 0.89-0.97; p < 0.01) and an intra-rater Cronbach's alpha coefficient of 0.96 (95% CI: 0.83-0.96; p < 0.01). The total score presented a negative correlation with forced expiratory volume in one second in liters (r = −0.49; p < 0.05) and with distance covered on the 6MWT (r = −0.56; p < 0.05). Conclusion: The Brazilian Portuguese version of the LCADL scale is a reliable, reproducible, and valid instrument for evaluating dyspnea during activities of daily living in patients with severe COPD.

Keywords: Activities of daily living; Dyspnea; Diagnostic techniques and procedures; Reproducibility of results.

Abstract Objective: To evaluate whether respiratory therapy techniques influence the number of cells within and quantity of induced sputum in patients with asthma or chronic obstructive pulmonary disease (COPD). Methods: Randomized clinical trial, in which patients with asthma or COPD under intervention (n = 16 and 10, respectively) were compared with control groups (n = 16 and 10). Patients in the asthma/intervention (A/I) and COPD/intervention (C/I) groups were submitted to oscillating positive expiratory pressure maneuvers for 5 min, followed by 10 forced expiratory technique sequences. These patients were also submitted to an induced sputum protocol with inhaled hypertonic saline (3%, 4% or 5%; A/I group) or inhaled isotonic saline (C/I group). The asthma/control (A/C) and COPD/control (C/C) groups were submitted only to the standard induced sputum protocol. Results: The final mean weight of the sputum samples was significantly greater in the A/I group than in the A/C group (2,767.25 ± 998.08 mg vs. 1,689.17 ± 1,189.96 mg; p = 0.03). The mean/median total cell counts (×106/mL) were higher in the A/I and C/I groups than in the A/C and C/C groups (4.06/0.95 and 0.63/0.39, p = 0.05, vs. 5.08/1.77 and 0.64/0.40, p = 0.02). There were no statistically significant differences among the groups in terms of cell viability. Conclusions: The use of respiratory therapy techniques can increase sputum sample weight in asthma patients, as well as increasing total cell counts in patients with asthma or COPD.

Keywords: Asthma; Pulmonary disease, obstructive chronic; Sputum; Physical therapy modalities.

Objective: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF) and whether pulmonary deposition correlates with disease severity or genotype. Methods: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25%. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low-energy all-purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1). One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2). Results: We included 24 patients (12 males) aged 5-27 years (mean ± SD: 12.85 ± 6.64 years). The Shwachman score (SS) was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were ΔF508 homozygotes, 13 were ΔF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017) and in heterozygotes (p = 0.043). Conclusions: The use of a respiratory therapy technique and the administration of inhaled albuterol immediately prior to the use of inhaled tobramycin decreased the pulmonary deposition of the latter in CF patients, and this reduction correlates with disease severity and genotype.

Keywords: Cystic fibrosis; Tobramycin; Respiratory therapy; Albuterol; Radionuclide imaging.

Objective: To study the clinical, radiological, and histopathological patterns of transbronchial biopsy (TBB) used in order to confirm the diagnosis in patients with clinical suspicion of interstitial lung disease (ILD) treated at a tertiary-care university hospital. Methods: We reviewed the medical records, radiology reports, and reports of transbronchial biopsies from all patients with suspected ILD who underwent TBB between January of 1999 and December of 2006 at the Hospital das Clínicas de Botucatu, located in the city of Botucatu, Brazil. Results: The study included 56 patients. Of those, 11 (19.6%) had a definitive diagnosis of idiopathic pulmonary fibrosis (IPF), the rate of which was significantly higher in the patients in which ILD was a possible diagnosis in comparison with those in which ILD was the prime suspect (p = 0.011), demonstrating the contribution of TBB to the diagnostic confirmation of these diseases. The histopathological examination of the biopsies revealed that 27.3% of the patients with IPF showed a pattern of organizing pneumonia, which suggests greater disease severity. The most common histological pattern was the indeterminate pattern, reflecting the peripheral characteristic of IPF. However, the fibrosis pattern showed high specificity and high negative predictive value. For CT scan patterns suggestive of IPF, the ROC curve showed that the best relationship between sensitivity and specificity occurred when five radiological alterations were present. Honeycombing was found to be strongly suggestive of IPF (p = 0.01). Conclusions: For ILDs, chest CT should always be performed, and TBB should be used in specific situations, according to the suspicion and distribution of lesions.

Keywords: Lung diseases, interstitial; Diagnosis, differential; Bronchoscopy.

Objective: The objective of this study was to evaluate the applicability of a modified version of the Saint George's Respiratory Questionnaire. The version evaluated elicits "agree" and "do not agree", rather than "yes" and "no", responses. The intention is to facilitate the comprehension of double-negative questions and to promote better recollection of symptoms by patients by shortening their symptom histories from 12 months to 3 months. Methods: A total of 30 clinically stable patients with chronic obstructive pulmonary disease were evaluated. The Saint George's Respiratory Questionnaire and the modified version of the same were administered 15 days apart. Results: All of the patients presented health-related alterations in their quality of life. Comparing mean scores between the two questionnaires, the greatest difference was seen in the Symptoms domain. No significant differences were found in any of the remaining domains or in the total scores. In a subsequent analysis, significant correlations between the two questionnaires were found in all domains: Symptoms (r = 0.71; p < 0.001); Activity (r = 0.75; p < 0.001); Impact (r = 0.73; p < 0.001) and Total (r = 0.86; p < 0.001). Conclusion: The modified version of the Saint George's Respiratory Questionnaire is as effective as the original in gauging quality of life. However, various symptoms recollection time frames should be investigated in order to determine which would be the best time frame to employ in the analysis.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Reproducibility of results; Cross-cultural comparison; Questionnaires