Samia Zahi Rached

Comparison between objective measures of smoking and self-reported smoking status in patients with asthma or COPD: are our patients telling us the truth?

OBJECTIVE: Smoking prevalence is frequently estimated on the basis of self-reported smoking status. That can lead to an underestimation of smoking rates. The aim of this study was to evaluate the difference between self-reported smoking status and that determined through the use of objective measures of smoking at a pulmonary outpatient clinic. METHODS: This was a cross-sectional study involving 144 individuals: 51 asthma patients, 53 COPD patients, 20 current smokers, and 20 never-smokers. Smoking status was determined on the basis of self-reports obtained in interviews, as well as through tests of exhaled carbon monoxide (eCO) and urinary cotinine. RESULTS: All of the asthma patients and COPD patients declared they were not current smokers. In the COPD and asthma patients, the median urinary cotinine concentration was 167 ng/mL (range, 2-5,348 ng/mL) and 47 ng/mL (range, 5-2,735 ng/mL), respectively (p < 0.0001), whereas the median eCO level was 8 ppm (range, 0-31 ppm) and 5 ppm (range, 2-45 ppm), respectively (p < 0.05). In 40 (38%) of the patients with asthma or COPD (n = 104), there was disagreement between the self-reported smoking status and that determined on the basis of the urinary cotinine concentration, a concentration > 200 ng/mL being considered indicative of current smoking. In 48 (46%) of those 104 patients, the self-reported non-smoking status was refuted by an eCO level > 6 ppm, which is also considered indicative of current smoking. In 30 (29%) of the patients with asthma or COPD, the urinary cotinine concentration and the eCO level both belied the patient claims of not being current smokers. CONCLUSIONS: Our findings suggest that high proportions of smoking pulmonary patients with lung disease falsely declare themselves to be nonsmokers. The accurate classification of smoking status is pivotal to the treatment of lung diseases. Objective measures of smoking could be helpful in improving clinical management and counseling.

Predicting high risk of exacerbations in bronchiectasis: the E-FACED score

Background Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. Objective Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. Methods The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. Results A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P<0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. Conclusion E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the score’s simplicity and prognostic capacity for death.

Incremental Shuttle Walking Test: A Reproducible and Valid Test to Evaluate Exercise Tolerance in Adults With Noncystic Fibrosis Bronchiectasis

OBJECTIVE To analyze the reliability, validity, and determinants of the incremental shuttle walk test (ISWT) in adults with noncystic fibrosis bronchiectasis. DESIGN Cross-sectional study. SETTING Outpatient clinic. PARTICIPANTS Subjects (N=75; 26 men) underwent, on different days, cardiopulmonary exercise testing (CPET) and 2 ISWTs, 30 minutes apart. The number of steps in daily life was recorded. Concurrent validity was tested by the relation between distance walked with peak load and oxygen consumption (V˙o2). INTERVENTIONS None. MAIN OUTCOME MEASURES Distance walked (m) was compared between the first and second ISWTs; greatest distance walked was correlated with peak load and Vo2peak obtained from CPET, steps per day, and dyspnea evaluated by the Medical Research Council (MRC) scale; and desaturation was compared between CPET and the ISWT. RESULTS Distance walked was equivalent between the first ISWT (441±152m) and the second ISWT (445±153m) with an excellent intraclass correlation coefficient (.995; 95% confidence interval, .99-.997). There were significant correlations between distance walked and peak load (r=.82), V˙o2 (r=.72), steps per day (r=.61), and the MRC scale (r=-.69). Age, body mass index, sex, forced vital capacity (% predicted), dyspnea, and steps per day explained 70% of the variation in distance walked (m) and 60% of the variance when expressed as percent predicted. Higher desaturation was observed during the ISWT (-4%±4%) than cycling (-2±3%) (P<.001). CONCLUSIONS The ISWT is reliable, represents functional capacity, and induces greater desaturation than cycling. Age, body composition, pulmonary function, dyspnea, and physical activity in daily life are determinants of the distance walked on the ISWT.

Deve-se extrapolar o tratamento de bronquiectasias em pacientes com fibrose cística para aqueles com bronquiectasias de outras etiologias?

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.

Corticoide sistêmico como tratamento de primeira linha da hipertensão pulmonar secundária a síndrome POEMS

The POEMS syndrome is a rare plasma cell disease. Pulmonary hypertension is an infrequent respiratory complication of this syndrome and might be associated with increased levels of various cytokines, chemokines and growth factors as part of the inflammatory phenomena that involve the physiopathology of POEMS syndrome. We present the case of a 54-year-old woman diagnosed with POEMS syndrome and pulmonary hypertension, which were treated with corticosteroids as the first-line therapy. The patient presented with the classic symptoms of this syndrome: polyneuropathy (confirmed by electromyography), organomegaly, subclinical hypothyroidism and monoclonal gammopathy detected in urine, together with skin changes. Right heart catheterization revealed a mean pulmonary artery pressure of 48 mmHg, a cardiac output of 4.1 L/min and pulmonary vascular resistance of 8.05 Woods. The serum level of brain natriuretic peptide (BNP) was 150 pg/mL. No other underlying disease was found during the investigation. Prednisone (1 mg/kg for three months) was then initiated, with a dramatic improvement in the clinical and functional condition. Levels of thyroid hormones and urinary protein levels (as determined using electrophoresis) normalized. Mean pulmonary artery pressure decreased to 26 mmHg, cardiac output decreased to 3.8 L/min, and pulmonary vascular resistance decreased to 2.89 Woods. Serum levels of BNP dropped to 8 pg/mL. Our findings suggest that corticosteroids could play a role as a first-line treatment in pulmonary hypertension accompanied by POEMS syndrome. Due to the rarity of this presentation, a multicenter registry should be developed to allow the compilation of additional data to support this practice.

Latin America validation of FACED score in patients with bronchiectasis: an analysis of six cohorts

BackgroundThe FACED score is an easy-to-use multidimensional grading system that has demonstrated an excellent prognostic value for mortality in patients with bronchiectasis. A Spanish group developed the score but no multicenter international validation has yet been published.MethodsRetrospective and multicenter study conducted in six historical cohorts of patients from Latin America including 651 patients with bronchiectasis. Clinical, microbiological, functional, and radiological variables were collected, following the same criteria used in the original FACED score study. The vital status of all patients was determined in the fifth year of follow-up. The area under ROC curve (AUC-ROC) was used to calculate the predictive power of the FACED score for all-cause and respiratory deaths and both number and severity of exacerbations. The discriminatory power to divide patients into three groups of increasing severity was also analyzed.ResultsMean (SD) age of 48.2 (16), 32.9% of males. The mean FACED score was 2.35 (1.68). During the follow up, 95 patients (14.6%) died (66% from respiratory causes). The AUC ROC to predict all-cause and respiratory mortality were 0.81 (95% CI: 0.77 to 0.85) 0.84 (95% CI: 0.80 to 0.88) respectively, and 0.82 (95% CI: 078–0.87) for at least one hospitalization per year. The division into three score groups separated bronchiectasis into distinct mortality groups (mild: 3.7%; moderate: 20.7% and severe: 48.5% mortality; p < 0.001).ConclusionsThe FACED score was confirmed as an excellent predictor of all-cause and respiratory mortality and severe exacerbations, as well as having excellent discriminative capacity for different degrees of severity in various bronchiectasis populations.

Does home-based pulmonary rehabilitation improve functional capacity, peripheral muscle strength and quality of life in patients with bronchiectasis compared to standard care?

Highlights • Home-based pulmonary rehabilitation (HBPR) has been used in several chronic pulmonary obstructive diseases.• HBPR has never been investigated in patients with bronchiectasis.• Short- and long-term effects of HBPR will be investigated in this population.• The study will provide evidence to guide recommendations about HBPR for bronchiectasis.

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis

A fibrose cistica (FC) e uma doenca genetica autossomica recessiva caracterizada pela disfuncao do gene CFTR. Trata-se de uma doenca multissistemica que ocorre mais frequentemente em populacoes descendentes de caucasianos. Nas ultimas decadas, diversos avancos no diagnostico e tratamento da FC mudaram drasticamente o cenario dessa doenca, com aumento expressivo da sobrevida e qualidade de vida. Atualmente, o Brasil dispoe de um programa de ampla cobertura para a triagem neonatal de FC e centros de referencia distribuidos na maior parte desses estados para seguimento dos individuos. Antigamente confinada a faixa etaria pediatrica, tem-se observado um aumento de pacientes adultos com FC tanto pelo maior numero de diagnosticos de formas atipicas, de expressao fenotipica mais leve, assim como pelo aumento da expectativa de vida com os novos tratamentos. Entretanto, ainda se observa uma grande heterogeneidade no acesso aos metodos diagnosticos e terapeuticos para FC entre as diferentes regioes brasileiras. O objetivo dessas diretrizes foi reunir as principais evidencias cientificas que norteiam o manejo desses pacientes. Um grupo de 18 especialistas em FC elaborou 82 perguntas clinicas relevantes que foram divididas em cinco categorias: caracteristicas de um centro de referencia; diagnostico; tratamento da doenca respiratoria; tratamento gastrointestinal e nutricional; e outros aspectos. Diversos profissionais brasileiros atuantes na area da FC foram convidados a responder as perguntas formuladas pelos coordenadores. A literatura disponivel foi pesquisada na base de dados PubMed com palavras-chave, buscando-se as melhores respostas as perguntas dos autores.

Reduced Physical Activity With Bronchiectasis

BACKGROUND: Bronchiectasis leads to reduced functional capacity, which might have implications for physical activity. The impact of dyspnea and long-term oxygen therapy on physical activity has never been investigated in subjects with bronchiectasis. Based on these findings, specific strategies could be applied to allow individuals to be more active in their daily life. In this study we aimed to evaluate physical activity, the impact of dyspnea and long-term oxygen therapy on physical activity, and the determinants of physical activity in subjects with bronchiectasis. METHODS: We performed a cross-sectional study in 139 subjects with bronchiectasis (age 45 ± 13 y, FVC 70 ± 22% of predicted, FEV1 54 ± 25% of predicted) and 49 healthy subjects as controls. Physical activity was assessed using steps per day (measured with a pedometer), spirometry, incremental shuttle walking test (ISWT), and dyspnea. RESULTS: Reduced physical activity was observed in subjects with bronchiectasis, who recorded a median (interquartile range) of 8,007 (5,131–10,432) steps/d compared with controls, who recorded 10,994 (8,551–14,078) steps/d (P < .001). Significant correlations were observed between physical activity and FVC (r = 0.43), FEV1 (r = 0.36), ISWT (r = 0.37), and dyspnea (r = −0.48). Determinants for reduced physical activity included pulmonary function (R2 = 0.150), dyspnea (R2 = 0.075), ISWT (R2 = 0.044), and long-term oxygen therapy (R2 = 0.038); these factors explained 32% of the physical activity. CONCLUSION: Subjects with bronchiectasis exhibited reduced physical activity compared with healthy peers. Dyspnea has a negative impact on physical activity. Independent factors associated with physical activity included pulmonary function, dyspnea, functional capacity, and long-term oxygen therapy. These findings will guide strategies to enhance daily physical activity and to encourage subjects with bronchiectasis to be more active.

The annual prognostic ability of FACED and E-FACED scores to predict mortality in patients with bronchiectasis

Bronchiectasis is a disease defined by a permanent and usually progressive bronchial dilation associated with multiple exacerbations and decreased health-related quality of life [1–3]. Improvement in the current knowledge of this conditions pathophysiology has clearly highlighted its complex and heterogeneous profile, whose severity or prognosis cannot be defined using a single variable [4]. Accordingly, multidimensional scores including demographical, clinical, microbiological and radiological data have recently been developed and validated as useful tools to better evaluate the diseases severity and prognosis: FACED (forced expiratory volume in 1 s (FEV1), age, chronic colonisation by Pseudomonas aeruginosa, radiological extension and dyspnoea), E-FACED (FACED plus exacerbations) and the bronchiectasis severity index (BSI) [5–8]. Both FACED and E-FACED scores have shown good short-term prognostic value for predicting mortality in bronchiectasis http://ow.ly/albl30i11bv