Objective: To profile the characteristics of adult patients with bronchiectasis, drawing comparisons between cystic fibrosis (CF) patients and those with bronchiectasis from other causes in order to determine whether it is rational to extrapolate the bronchiectasis treatment given to CF patients to those with bronchiectasis from other causes. Methods: A retrospective analysis of the medical charts of 87 patients diagnosed with bronchiectasis and under follow-up treatment at our outpatient clinic. Patients who had tuberculosis (current or previous) were excluded. We evaluated the clinical, functional, and treatment data of the patients. Results: Of the 87 patients with bronchiectasis, 38 (43.7%) had been diagnosed with CF, through determination of sweat sodium and chloride concentrations or through genetic analysis, whereas the disease was due to another etiology in 49 (56.3%), of whom 34 (39.0%) had been diagnosed with idiopathic bronchiectasis. The mean age at diagnosis was lower in the patients with CF than in those without (14.2 vs. 24.2 years; p < 0.05). The prevalence of symptoms (cough, expectoration, hemoptysis, and wheezing) was similar between the groups. Colonization by Pseudomonas aeruginosa or Staphylococcus aureus was more common in the CF patients (82.4 vs. 29.7% and 64.7 vs. 5.4%, respectively). Conclusions: The causes and clinical manifestations of bronchiectasis are heterogeneous, and it is important to identify the differences. It is crucial that these differences be recognized so that new strategies for the management of patients with bronchiectasis can be developed.

Keywords: Cystic fibrosis; Bronchiectasis/diagnosis; Bronchiectasis/therapy; Respiratory function tests.

Objectives: To evaluate the self-reported degree of adherence to treatment in patients with cystic fibrosis (CF), investigating associations with characteristics of the disease and with the degree of adherence perceived by health professionals. Methods: This was a prospective, cross-sectional study involving patients with CF monitored at a Program for Adults with CF. The degree of adherence was evaluated using a questionnaire. Patients were divided into two groups: greater degree of adherence and moderate/poor degree of adherence. Clinical data, Shwachman-Kulczycki clinical score, Brasfield radiographic score and spirometry data were obtained for all patients. Results: Out of 38 patients studied, 31 (81.6%) were classified as presenting a greater degree of adherence and 7 (18.4%) as presenting a moderate/poor degree of adherence. The self-reported patient adherence score correlated with the clinical score (r = −0.36, p = 0.028). The self-reported patient adherence score (median = 0.79) was higher than that perceived by health professionals (median = 0.71, p = 0.003). A greater degree of adherence was self-reported for respiratory therapy (by 84.2%), exercise (by 21.1%), prescribed diet (by 65.8%), pancreatic enzymes (by 96.3%), vitamins (by 79.4%), inhaled antibiotic therapy (by 76.7%) and inhaled DNase (by 79.4%). Conclusions: Self-reported adherence of patients attending a Program for Adults with CF was good. The self-reported patient adherence score correlated inversely with the clinical score. Self-reported patient adherence was greater than the adherence perceived by health professionals.

Keywords: Cystic fibrosis; Patient compliance; Therapeutics.

Objective: To evaluate physical performance on the six-minute walk test (6MWT) in patients with non-cystic fibrosis bronchiectasis and to investigate its relationship with quality of life (QoL). To identify predictors of exercise performance, we also investigated whether six-minute walk distance (6MWD) is associated with clinical and spirometric findings. Methods: This was a cross-sectional study involving patients with non-cystic fibrosis bronchiectasis (age,  18 years), with at least one respiratory symptom for  2 years and an FEV1  70% of predicted. Patients underwent clinical evaluation, pulmonary function tests, the 6MWT, and QoL assessment with the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). Results: We included 70 patients (48 females). Mean age was 54.5 ± 17.7 years, and mean FEV1 was 44.9 ± 14.5% of predicted. The patients were divided into two groups: 6MWD-low (6MWD below the predicted lower limit; n = 23); and 6MWD-norm (normal 6MWD; n = 47). The following variables were significantly lower in the 6MWD-low group than in the 6MWD-norm group: age; age at diagnosis of bronchiectasis; proportion of former smokers; body mass index (BMI); FEV1% of predicted; and MEP% of predicted. There were no significant differences in the SF-36 scores between the groups. In the logistic regression model, lower age and lower BMI were significantly associated with lower 6MWD. Conclusions: In this sample, there was a high proportion of patients who had a lower than expected 6MWD. Although 6MWD was not related to QoL, it was associated with age and BMI.

Keywords: Bronchiectasis; Quality of life; Respiratory function tests; Exercise tolerance.

Objectives: This study primarily aimed to investigate the clinical determinants of the Modified Incremental Step Test (MIST) in adults with non-cystic fibrosis bronchiectasis (NCFB). A secondary objective was to compare the cardiopulmonary responses after the MIST and Incremental Shuttle Walk Test (ISWT), two commonly adopted symptom-limited maximum field tests in chronic respiratory diseases. Methods: Forty-six patients with clinically stable bronchiectasis participated in this cross-sectional study. MIST and ISWT were performed to determine exercise capacity, while disease severity, fatigue, and quality of life were assessed using the Bronchiectasis Severity Index (BSI), the Fatigue Severity Scale (FSS), and St. George's Respiratory Questionnaire (SGRQ), respectively. Quadriceps muscle strength was evaluated using a hand-held dynamometer, walking speed with a wireless inertial sensing device, and the level of physical activity (steps/day) with a pedometer. Results: The BSI score, quadriceps muscle strength, daily step count, and the SGRQ total score explained 61.9% of the variance in the MIST (p < 0.001, R2 = 0.67, AR2 = 0.619). The BSI score (r = -0.412, p = 0.004), quadriceps muscle strength (r = 0.574, p = 0.001), daily step count (r = 0.523, p < 0.001), walking speed (r = 0.402, p = 0.006), FSS score (r = -0.551, p < 0.001), and SGRQ total score (r = -0.570, p < 0.001) correlated with the MIST. The patients achieved higher heart rates (HR), HR%, desaturation, dyspnea, and leg fatigue in the MIST compared to the ISWT (p < 0.05). Conclusions: Disease severity, quadriceps muscle strength, physical activity level, and quality of life were determinants of MIST. The advantages of the MIST, including higher cardiopulmonary response than ISWT and greater portability, which facilitates its use in various settings, make MIST the preferred choice for investigating symptom-limited exercise capacity in patients with NCFB.

Keywords: bronchiectasis, exercise capacity, step test, physical activity, walking speed, quality of life.

Objective: To identify microorganisms in sputum samples of patients with stable non-cystic fibrosis bronchiectasis and to determine risk factors related to the isolation of Pseudomonas aeruginosa (PA) in those patients. Methods: Consecutive patients were recruited from a tertiary hospital outpatient clinic in the city of Fortaleza, Brazil. The patients were submitted to spirometry, six-minute walk test, HRCT, and sputum collection. Data on serum fibrinogen levels, disease severity, sputum color, and history of azithromycin treatment were collected. Results: The study included 112 patients, and females predominated (68%). The mean age was 51.6 ± 17.4 years. Most patients presented with mild-to-moderate disease (83%). The mean six-minute walk distance was 468.8 ± 87.9 m. Mean FEV1 and FVC, in % of predicted values, were 60.4 ± 21.8% and 69.9 ± 18.5%, respectively. The mean serum fibrinogen level was 396.1 ± 76.3 mg/dL. PA was isolated in 47 patients, other potentially pathogenic microorganisms (PPMs) were isolated in 31 patients, and non-PPMs were isolated in 34 patients. Purulent sputum was identified in 77 patients (68%). The patients with PA, when compared with those without it, presented with more severe disease, higher serum fibrinogen levels, and lower FVC%. In addition, purulent sputum and long-term azithromycin treatment were more common in those with PA. The multivariate regression analysis showed that the independent factors associated with PA were serum fibrinogen level > 400 mg/dL (OR = 3.0; 95% CI: 1.1-7.7) and purulent sputum (OR = 4.3; 95% CI: 1.6-11.3). Conclusions: In our sample, the prevalence of PA in sputum was 42%. Sputum color and inflammatory markers were able to predict the isolation of PA, emphasizing the importance of routine sputum monitoring.

Keywords: Bronchiectasis; Pseudomonas aeruginosa; Sputum/microbiology.

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

Keywords: Bronchiectasis; Tomography, X-ray; Radiography, thoracic.

Objective: The frequency of obstructive sleep apnea (OSA) in patients with idiopathic pulmonary fibrosis (IPF) is high. The clinical course of non-IPF interstitial lung disease (ILD) can be similar to that of IPF. We sought to assess the frequency and predictors of OSA in patients with non-IPF fibrotic ILD, as well as the impact of positive airway pressure (PAP) therapy on the quality of life of such patients. Methods: This was a prospective study in which non-IPF fibrotic ILD patients underwent a home sleep apnea test. The patients with and without OSA were compared, and a multivariate logistic regression model was used to identify independent predictors of OSA. At 3 months after initiation of PAP therapy, we evaluated the participating patients for respiratory events, nocturnal hypoxemia, and changes in quality of life. Results: Of a total of 50 patients, 50% were male, and 76% were diagnosed with OSA. The mean age was 67.8 ± 8.3 years. The patients with OSA had significantly lower TLC (p = 0.033) and awake SpO2 (p = 0.023) than did those without OSA. In the multivariate logistic regression model, SpO2 (OR = 0.46; p = 0.016) and TLC (OR = 0.95; p = 0.026) remained significantly associated with OSA risk. A total of 12 patients received PAP therapy. At 3 months after initiation of PAP therapy, 91.7% were well controlled, Epworth Sleepiness Scale scores decreased significantly (p = 0.006), and emotional well-being tended to improve (p = 0.068). PAP therapy corrected nocturnal hypoxemia in all patients. Conclusions: We found a high frequency of OSA in patients with non-IPF fibrotic ILD. A low TLC was an independent predictor of a higher risk of OSA. PAP therapy can correct nocturnal hypoxemia. There should be a low threshold for suspicion of OSA and initiation of PAP therapy in patients with non-IPF fibrotic ILD.

Keywords: Sleep apnea, obstructive; Fibrosis, pulmonary; Total lung capacity; Hypoxia; Quality of life.

Objective: To evaluate the effect that the distribution of food baskets to tuberculosis (TB) patients has on treatment outcomes at a primary health care clinic. Methods: Retrospective comparative study of the medical and social aspects of 142 patients at a primary health care clinic in the city of Duque de Caxias, Brazil. The patients were divided into two groups: the first group included 68 patients treated with standard regimens (between September of 2001 and December of 2003); and the second group included 74 patients treated with the same regimens but also receiving food baskets on a monthly basis (between January of 2004 and July of 2006). Results: The statistical comparison between the two groups revealed that the cure rate was higher in the group receiving the food baskets (87.1% vs. 69.7%), whereas the rate of noncompliance was markedly lower (12.9% vs. 30.3%). Conclusions: The results indicate that the distribution of food baskets can be a useful strategy to improve compliance with TB treatment at primary health care clinics.

Keywords: Tuberculosis; Nutritional support; Treatment outcome; Ambulatory health services.

Objective: Surgical resection remains the gold standard treatment for bronchiectasis in patients who present with hemoptysis or suppuration, as well as in those who do not respond to clinical treatment. We sought to investigate the efficacy of sublobar resection (segmentectomy) and compare it with that of lobar resection (lobectomy) in patients with non-cystic fibrosis bronchiectasis. Methods: Patients undergoing lobectomy or segmentectomy between 2019 and 2023 were included in the study. We analyzed intraoperative complications and postoperative outcomes, including length of hospital stay, length of ICU stay, and disease recurrence. Results: There was no significant difference between the lobectomy and segmentectomy groups regarding the occurrence of intraoperative complications such as bleeding > 1000 ml, cardiogenic shock, and ventilatory instability (p > 0.999). However, the frequency of complications was significantly lower in the segmentectomy group than in the lobectomy group (p = 0.016). Hospital stays were longer in the lobectomy group than in the segmentectomy group (16 days vs. 5 days; p = 0.027), as were ICU stays (7 days vs. 1 day; p = 0.006). There was no significant difference between the lobectomy and segmentectomy groups regarding the recurrence rate (p = 0.541). Conclusions: Early identification of bronchiectasis patients who are candidates for surgical resection is essential because those who are identified as such early on are candidates for parenchyma-sparing resections, which are similar to lobar resections in terms of disease control and lead to shorter hospital stays and better postoperative outcomes.

Keywords: Bronchiectasis; Thoracic surgery; Disease-free survival.

Different kinds of pulmonary impairment have been described in Sjögren syndrome, including rare cases of bronchiectasis. The authors report a female patient with a history of episodes of respiratory infections and progressive breathlessness whose high resolution computerized tomography revealed bronchiectasis. A former open lung biopsy showed bronchiolar inflammatory and fibrotic changes. The diagnosis of Sjögren syndrome was made only late in the evolution, although sicca syndrome symptoms had been present for years. The authors discuss the potential pathogenic mechanisms involved in the development of the bronchiectasis and the need for a high degree of clinical medical skill for the early diagnosis of such conditions.

Keywords: Sjögren syndrome; bronchiectasis; bronchiolitis

Objetivo: Medir a resistência de vias aéreas utilizando a técnica de resistência do interruptor (Rint) em pacientes com fibrose cística (FC) e correlacioná-la com parâmetros espirométricos, assim como avaliar a acurácia de Rint para determinar a resposta das vias aéreas a um broncodilatador. Métodos: Estudo transversal com 38 crianças e adolescentes com FC acompanhados no Ambulatório de FC do Hospital São Lucas, em Porto Alegre (RS). Após a determinação de Rint, os pacientes foram submetidos à espirometria. Para a avaliação da resposta ao broncodilatador, as medições foram repetidas após o uso de salbutamol inalatório. Resultados: Houve uma forte correlação entre o inverso de Rint e VEF1 (r = 0,8; p < 0,001) e moderadas correlações entre o inverso de Rint e FEF25-75% (r = 0,74; p < 0,001) e entre o inverso de Rint e índice de massa corpórea (r = 0,62; p < 0,001). A curva ROC foi utilizada na comparação da resposta ao broncodilatador determinada por Rint com aquela determinada por valores espirométricos. Para um ponto de corte de −28% para Rint, a área sob a curva foi de 0,75, com uma sensibilidade de 66% e uma especificidade de 82%. Conclusões: Nossos achados indicam que Rint apresenta uma boa correlação com parâmetros espirométricos, embora a técnica Rint não tenha sido suficientemente acurada para substituir a espirometria na avaliação da resposta ao broncodilatador.

Keywords: Testes de função respiratória; Fibrose cística; Espirometria; Resistência das vias respiratórias.

Objective: To compare two methods of respiratory inductive plethysmography (RIP) calibration in three different positions. Methods: We evaluated 28 healthy subjects (18 women and 10 men), with a mean age of 25.4 ± 3.9 years. For all of the subjects, isovolume maneuver calibration (ISOCAL) and qualitative diagnostic calibration (QDC) were used in the orthostatic, sitting, and supine positions. In order to evaluate the concordance between the two calibration methods, we used ANOVA and Bland-Altman plots. Results: The values of the constant of proportionality (K) were significantly different between ISOCAL and QDC in the three positions evaluated: 1.6 ± 0.5 vs. 2.0 ± 1.2, in the supine position, 2.5 ± 0.8 vs. 0.6 ± 0.3 in the sitting position, and 2.0 ± 0.8 vs. 0.6 ± 0.3 in the orthostatic position (p < 0.05 for all). Conclusions: Our results suggest that QDC is an inaccurate method for the calibration of RIP. The K values obtained with ISOCAL reveal that RIP should be calibrated for each position evaluated.

Keywords: Plethysmography; Respiratory mechanics; Posture; Calibration.

Objective: Obstructive sleep apnea (OSA) is a common disorder associated with a significant economic burden. Continuous positive airway pressure (CPAP) and auto-titrating positive airway pressure (APAP) are recognized therapeutic options in patients with OSA, although treatment costs are higher with APAP. We conducted a study aimed at evaluating the effectiveness and potential cost savings resulting from the implementation of a protocol guiding the transition to CPAP in OSA patients previously treated with APAP. Methods: This prospective study included patients with OSA under APAP who were followed up at the Sleep Medicine outpatient clinic of a tertiary referral hospital between January 2019 and January 2021. Treatment was switched to CPAP in patients who met the following criteria: satisfactory adaptation and adherence to APAP, residual apnea-hypopnea index (AHI) of < 5/hour, and no relevant air leaks. APAP and CPAP outcomes were compared and an estimate of the savings obtained by the transition from APAP to CPAP was calculated. Results: Ninety-three patients were included in the study. APAP and CPAP were both effective in correcting obstructive events and improving daytime sleepiness. No significant differences were found regarding treatment adherence and tolerance between both PAP modalities. The selection of fixed-pressure CPAP through 90th or 95th percentile APAP pressure proved to be effective and an alternative strategy to titration polysomnography. At the end of this two-year study, the transition from APAP to CPAP enabled savings of at least 10,353?. Conclusion: The transition from APAP to CPAP may be an effective, well-tolerated, safe, and cost-saving strategy in patients with OSA.

Keywords: Obstructive sleep apnea (OSA); continuous positive airway pressure (CPAP); auto-titrating positive airway pressure (APAP); health care costs

Objective: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. Methods: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12(6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Zscore, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1%, and FEF25-75%). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. Results: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. Conclusions: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.

Keywords: Cystic fibrosis; Spirometry; Body mass index; Physical therapy modalities.

Objective: To investigate the validity of field walking tests to identify exercise-induced hypoxemia and to compare cardiorespiratory responses and perceived effort between laboratory-based and field-based exercise tests in subjects with bronchiectasis. Methods: This was a cross-sectional study involving 72 non-oxygen-dependent participants (28 men; mean age = 48.3 ± 14.5 years; and mean FEV1 = 54.1 ± 23.4% of the predicted value). The participants underwent cardiopulmonary exercise testing (CPET) on a treadmill and constant work-rate exercise testing (CWRET) on the same day (1 h apart). In another visit, they underwent incremental shuttle walk testing (ISWT) and endurance shuttle walk testing (ESWT; 1 h apart). Desaturation was defined as a reduction in SpO2 ≥ 4% from rest to peak exercise. Results: CPET results were compared with ISWT results, as were CWRET results with ESWT results. There was no difference in the magnitude of desaturation between CPET and ISWT (−7.7 ± 6.3% vs. −6.6 ± 5.6%; p = 0.10) and between CWRET and ESWT (−6.8 ± 5.8% vs. −7.2 ± 6.3%; p = 0.50). The incremental tests showed an agreement in the magnitude of desaturation in the desaturation and no desaturation groups (42 and 14 participants, respectively; p < 0.01), as did the endurance tests (39 and 16 participants; p < 0.01). The magnitude of desaturation was similar among the participants who did or did not reach at least 85% of the maximum predicted HR. Conclusions: Field exercise tests showed good precision to detect desaturation. Field tests might be an alternative to laboratory tests when the clinical question is to investigate exercise-induced desaturation in subjects with bronchiectasis.

Keywords: Bronchiectasis; Oxygen; Hypoxia; Exercise test.