Association between swallowing dynamics, tongue pressure and pulmonary function in patients with idiopathic pulmonary fibrosis.

BACKGROUND: Swallowing is a complex process that requires the coordination of muscles in the mouth, pharynx, larynx, and esophagus. Dysphagia occurs when a person has difficulty swallowing. In the case of subjects with respiratory diseases, the presence of oropharyngeal dysphagia potentially increases lung disease exacerbations, which can lead to a rapid decline in lung function. This study aimed to analyze the swallowing of patients with idiopathic pulmonary fibrosis (IPF). METHODS: Patients with IPF were evaluated using the Eating Assessment Tool (EAT-10), tongue pressure, the Timed Water Swallow Test (TWST), and the Test of Mastication and Swallowing Solids (TOMASS). The findings were related to dyspnea severity assessed by the modified Medical Research Counsil (mMRC) score; the nutritional status screened with Mini Nutritional Assessment (MNA) tool; and pulmonary function tests, specifically spirometry and measurement of the diffusing capacity for carbon monoxide (DLCO), the maximal inspiratory pressure (PImax), and the maximal expiratory pressure (PEmax). RESULTS: The sample consisted of 34 individuals with IPF. Those who exhibited swallowing modifications scored lower on the MNA than those who did not (9.6 ± 0.76 vs. 11.64 ± 0.41 points; mean difference 1.98 ± 0.81 points; p = 0.02). They also showed poorer lung function when considering the predicted force vital capacity (FVC; 81.5% ± 4.61% vs. 61.87% ± 8.48%; mean difference 19.63% ± 9.02%; p = 0.03). The speed of liquid swallowing was altered in 31of 34 of the evaluated subjects (91.1%). The number of liquid swallows correlated significantly with the forced expiratory volume in 1 s (FEV1)/FVC ratio (r = 0.3; p = 0.02). Solid eating and swallowing assessed with the TOMASS score correlated with lung function. The number of chewing cycles correlated negatively with PImax% predicted (r = -0.4; p = 0.0008) and PEmax% predicted (r = -0.3; p = 0.02). FVC% predicted correlated with increased solid swallowing time (r = -0.3; p = 0.02; power = 0.6). Swallowing solids was also impacted by dyspnea. CONCLUSION: Patients with mild-to-moderate IPF can present feeding adaptations, which can be related to the nutritional status, lung function, and the severity of dyspnea.

Use of fractional exhaled nitric oxide as a potential predictor of bronchodilator response.

Nitric oxide (NO) is an important product of eosinophilic metabolism, and its increase is associated with bronchial remodeling and airway hyperresponsiveness. Fractional exhaled NO (FENO) in the expired air of patients with suspected or diagnosed asthma has been used as a marker for eosinophilic inflammation. This cohort study included asthmatic patients classified under steps 3, 4, or 5 of the global strategy for asthma management and prevention. In the morning of the same day, all patients underwent blood collection for eosinophil counts, followed by FENO measurement and spirometry. We considered 2 groups based on the bronchodilation (BD) response on spirometry (>10% of FVC or FEV1): positive (BD+) and negative (BD-). Differences between the 2 groups were analyzed for demographic features, FENO values, and predictive correlations between FENO and BD. Both groups of patients showed an increase in the eosinophil count (BD+, P = .03; BD-, P = .04) and FENO values (P = .015 for both) with an increase in the asthma severity from step 3 to step 5 of the global strategy for asthma management and prevention. The correlations of FENO and eosinophils as well as FENO values and BD + were 0.127 (95% confidence interval,-0.269 to -0.486) and 0.696 (95% confidence interval, 0.246-0.899; P = .007), respectively. Measuring FENO levels may be useful for identifying patients with BD+.

Follow-up of a cohort of patients with noncystic fibrosis bronchiectasis for 1 year.

OBJECTIVE: The objective of this study was to evaluate the quality of life of patients with noncystic fibrosis bronchiectasis during a 1-year follow-up by using the EuroQol - 5 Dimensions - 3 Levels (EQ-5D-3L) questionnaire. METHODS: A cohort study was conducted with 100 patients with noncystic fibrosis bronchiectasis and followed up with face-to-face visits or by telephone contact every 3 months for 1 year. All patients were recruited from a single referral center for bronchiectasis. At the time of recruiting and at the end of 1 year, the EQ-5D-3L questionnaire was applied to evaluate the patients' quality of life. Variables, such as exacerbation, emergency care, comorbidities, hemoptysis, colonization, and hospitalization, were assessed. RESULTS: Of the 100 patients, 99 completed the study and 72% were women. There were no marked limitations in the mobility and self-care domains during the follow-up. At the end of the follow-up, 32 patients were extremely anxious or depressed. The quality of life assessed by using EQ-5D-3L had an initial mean score of 0.545 and of 0.589 after 1 year, which was statistically significant (p=0.011). CONCLUSION: Patients with noncystic fibrosis bronchiectasis have a poor quality of life, and the EQ-5D-3L questionnaire may be a tool for monitoring patients with bronchiectasis.

Follow-up of a cohort of patients with noncystic fibrosis bronchiectasis for 1 year

SUMMARY OBJECTIVE: The objective of this study was to evaluate the quality of life of patients with noncystic fibrosis bronchiectasis during a 1-year follow-up by using the EuroQol - 5 Dimensions - 3 Levels (EQ-5D-3L) questionnaire. METHODS: A cohort study was conducted with 100 patients with noncystic fibrosis bronchiectasis and followed up with face-to-face visits or by telephone contact every 3 months for 1 year. All patients were recruited from a single referral center for bronchiectasis. At the time of recruiting and at the end of 1 year, the EQ-5D-3L questionnaire was applied to evaluate the patients' quality of life. Variables, such as exacerbation, emergency care, comorbidities, hemoptysis, colonization, and hospitalization, were assessed. RESULTS: Of the 100 patients, 99 completed the study and 72% were women. There were no marked limitations in the mobility and self-care domains during the follow-up. At the end of the follow-up, 32 patients were extremely anxious or depressed. The quality of life assessed by using EQ-5D-3L had an initial mean score of 0.545 and of 0.589 after 1 year, which was statistically significant (p=0.011). CONCLUSION: Patients with noncystic fibrosis bronchiectasis have a poor quality of life, and the EQ-5D-3L questionnaire may be a tool for monitoring patients with bronchiectasis.

Mortality and comorbidities in patients with bronchiectasis over a 3-year follow-up.

To identify the risk factors associated with all-cause mortality in patients with noncystic fibrosis bronchiectasis (NCFB). This prospective cohort study included 120 adult patients with NCFB, who were regularly treated at a specialized outpatient clinic of a university hospital between January 2017 and June 2020. All patients were diagnosed using high-resolution computed tomography. Demographic and clinical data, pulmonary function tests, and the Euro-quality-of-life 5-domain 3-level questionnaire were analyzed. The factors associated with death were determined using the Cox proportional hazards model. The all-cause mortality rate at 41 months was 10.8%. Adjusted multivariate analysis showed that the main contributing predictors for mortality were female sex, smoking, diabetes, chronic obstructive pulmonary disease, emergency visits, use of antibiotics due to exacerbation, secretion color change, exacerbation, predicted forced expiratory volume in 1 second, predicted forced vital capacity, lack of respiratory physiotherapy, absence of vaccination against pneumococci, and mobility domain. Multiple factors contribute to unfavorable outcomes in patients with NCFB, and early recognition of these factors may improve care management.

The impact of healthcare-associated infections on COVID-19 mortality: a cohort study from a Brazilian public hospital.

OBJECTIVE: This study aims to analyze the risk factors for in-hospital mortality in a cohort of patients admitted to a newly adapted intensive care unit in a public hospital in Rio de Janeiro. METHODS: This was an observational, retrospective, and descriptive study. Data were obtained from electronic medical records. Coronavirus disease 2019 (COVID-19) was diagnosed by detecting viral ribonucleic acid using reverse transcription polymerase chain reaction. Factors associated with the risk/protection from death were determined using the odds ratio and adjusted odds ratio. RESULTS: Fifty-one patients were admitted to the hospital. The median age of the patients was 63 years, 60% were male patients, and 54% were white patients. Sixty-seven percent of the patients were diagnosed with COVID-19. Sepsis at admission increased the chance of in-hospital death by 21 times (adjusted odds ratio=21.06 [0.79-555.2]; p=0.06). The strongest risk factor for death was the development of septic shock during hospitalization (adjusted odds ratio=98.56 [2.75-352.5]; p=0.01), and one in four patients had multidrug-resistant bacteria. Mechanical ventilation, vasopressors, neuromuscular blockers, and sedatives were also the risk factors for in-hospital mortality. The in-hospital mortality rate was 41%, and the mortality rate of patients on mechanical ventilation was 60%. The diagnosis of COVID-19 was not statistically related to the adverse outcomes. CONCLUSIONS: In this cohort, the strongest risk factor for in-hospital death was the development of nosocomial septic shock. Healthcare-associated infections have a significant impact on mortality rates. Therefore, to have a better outcome, it is important to consider not only the availability of beds but also the way healthcare is delivered.

The impact of healthcare-associated infections on COVID-19 mortality: a cohort study from a Brazilian public hospital

SUMMARY OBJECTIVE: This study aims to analyze the risk factors for in-hospital mortality in a cohort of patients admitted to a newly adapted intensive care unit in a public hospital in Rio de Janeiro. METHODS: This was an observational, retrospective, and descriptive study. Data were obtained from electronic medical records. Coronavirus disease 2019 (COVID-19) was diagnosed by detecting viral ribonucleic acid using reverse transcription polymerase chain reaction. Factors associated with the risk/protection from death were determined using the odds ratio and adjusted odds ratio. RESULTS: Fifty-one patients were admitted to the hospital. The median age of the patients was 63 years, 60% were male patients, and 54% were white patients. Sixty-seven percent of the patients were diagnosed with COVID-19. Sepsis at admission increased the chance of in-hospital death by 21 times (adjusted odds ratio=21.06 [0.79-555.2]; p=0.06). The strongest risk factor for death was the development of septic shock during hospitalization (adjusted odds ratio=98.56 [2.75-352.5]; p=0.01), and one in four patients had multidrug-resistant bacteria. Mechanical ventilation, vasopressors, neuromuscular blockers, and sedatives were also the risk factors for in-hospital mortality. The in-hospital mortality rate was 41%, and the mortality rate of patients on mechanical ventilation was 60%. The diagnosis of COVID-19 was not statistically related to the adverse outcomes. CONCLUSIONS: In this cohort, the strongest risk factor for in-hospital death was the development of nosocomial septic shock. Healthcare-associated infections have a significant impact on mortality rates. Therefore, to have a better outcome, it is important to consider not only the availability of beds but also the way healthcare is delivered.

Defining prognosis in sarcoidosis.

Sarcoidosis is a multi-systemic granulomatous disease. Affected individuals can show spontaneous healing, develop remission with drug treatment within 2 years, or become chronically ill. Our main goal was to identify features that are related to prognosis.The study consisted of 101 patients, recruited at a single center, who were already diagnosed with sarcoidosis at the start of the study or were diagnosed within 48 months. Ninety individuals were followed-up for at least 24 months and were classified according to clinical outcome status (COS 1 to 9). Those with COS 1-4 and COS 5-9 were classified as having favorable and unfavorable outcomes, respectively. Unconditional logistic regression analyses were conducted to define which variables were associated with sarcoidosis outcomes. Subsequently, we established a scoring system to help predict the likelihood of a favorable or unfavorable outcome.Of our patients, 48% developed a chronic form of the disease (COS 5-9). Three clinical features were predictive of prognosis in sarcoidosis. We built a score-based model where the absence of rheumatological markers (1 point), normal pulmonary functions (2 points), and the presence of early respiratory symptoms manifestations (2 points) were associated with a favorable prognosis. We predicted that a patient with a score of 5 had an 86% (95% confidence interval [CI] 74%-98%) probability of having a favorable prognosis, while those with scores of 4, 3, 2, 1, and 0 had probabilities of 72% (95% CI 59-85%), 52% (95% CI 40-63%), 31% (95% CI 17-44%), 15% (95% CI 2-28%), and 7% (95% CI 0-16%) of having a favorable prognosis, respectively. Thus, our easy-to-compute algorithm can help to predict prognosis of sarcoidosis patients, facilitating their management.

Translation and cultural adaptation of the Sleep Apnea Clinical Score for use in Brazil.

OBJECTIVE: To translate the Sleep Apnea Clinical Score (SACS) into Brazilian Portuguese and adapt it to the cultural setting, validating it for use as a screening method for polysomnography and as a tool to quantify the risk of obstructive sleep apnea syndrome in individuals in Brazil. METHODS: The translation was performed by two professionals, with subsequent synthesis of the translations. From that version, a back-translation was prepared, revised, and compared with the original by a team of experts. As a pre-test, a consensus version was applied in 20 patients randomly selected from among those under treatment at outpatient clinics at the Piquet Carneiro Polyclinic of the State University of Rio de Janeiro, in the city of Rio de Janeiro, to assess their understanding of the questions. In the validation phase, the Brazilian-Portuguese version of the SACS was applied in 86 patients who subsequently underwent polysomnography, regardless of the SACS result. RESULTS: The analyses of the pre-test phase showed that the SACS was easily understood by the patients. In the validation phase, the SACS showed a sensitivity of 45.3% (95% CI: 32.8-58.2%), a specificity of 90.9% (95% CI: 70.8-98.9%), a positive predictive value of 93.5% (95% CI: 79.0-98.2%), a negative predictive value of 36.4% (95% CI: 30.6-42.5%), and an accuracy of 57.0% (95% CI: 45.8-67.6%). CONCLUSIONS: The Brazilian-Portuguese version of the SACS can be used in order to assess the risk of obstructive sleep apnea syndrome.

Brazilian guidelines for the pharmacological treatment of idiopathic pulmonary fibrosis. Official document of the Brazilian Thoracic Association based on the GRADE methodology.

Idiopathic pulmonary fibrosis (IPF) is a form of chronic interstitial lung disease of unknown cause, which predominantly affects elderly men who are current or former smokers. Even though it is an uncommon disease, it is of great importance because of its severity and poor prognosis. In recent decades, several pharmacological treatment modalities have been investigated for the treatment of this disease, and the classic concepts have therefore been revised. The purpose of these guidelines was to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of IPF in Brazil. We sought to provide guidance on the practical issues faced by clinicians in their daily lives. Patients of interest, Intervention to be studied, Comparison of intervention and Outcome of interest (PICO)-style questions were formulated to address aspects related to the use of corticosteroids, N-acetylcysteine, gastroesophageal reflux medications, endothelin-receptor antagonists, phosphodiesterase-5 inhibitors, pirfenidone, and nintedanib. To formulate the PICO questions, a group of Brazilian specialists working in the area was assembled and an extensive review of the literature on the subject was carried out. Previously published systematic reviews with meta-analyses were analyzed for the strength of the compiled evidence, and, on that basis, recommendations were developed by employing the Grading of Recommendations Assessment, Development and Evaluation approach. The authors believe that the present document represents an important advance to be incorporated in the approach to patients with IPF, aiming mainly to improve its management, and can become an auxiliary tool for defining public policies related to IPF.

Symptom variability over the course of the day in patients with stable COPD in Brazil: a real-world observational study.

OBJECTIVE: To analyze symptoms at different times of day in patients with COPD. METHODS: This was a multicenter, cross-sectional observational study conducted at eight centers in Brazil. We evaluated morning, daytime, and nighttime symptoms in patients with stable COPD. RESULTS: We included 593 patients under regular treatment, of whom 309 (52.1%) were male and 92 (15.5%) were active smokers. The mean age was 67.7 years, and the mean FEV1 was 49.4% of the predicted value. In comparison with the patients who had mild or moderate symptoms, the 183 (30.8%) with severe symptoms were less physically active (p = 0.002), had greater airflow limitation (p < 0.001), had more outpatient exacerbations (p = 0.002) and more inpatient exacerbations (p = 0.043), as well as scoring worse on specific instruments. The most common morning and nighttime symptoms were dyspnea (in 45.2% and 33.1%, respectively), cough (in 37.5% and 33.3%, respectively), and wheezing (in 24.4% and 27.0%, respectively). The intensity of daytime symptoms correlated strongly with that of morning symptoms (r = 0.65, p < 0.001) and that of nighttime symptoms (r = 0.60, p < 0.001), as well as with the COPD Assessment Test score (r = 0.62; p < 0.001), although it showed only a weak correlation with FEV1 (r = -0.205; p < 0.001). CONCLUSIONS: Dyspnea was more common in the morning than at night. Having morning or nighttime symptoms was associated with greater daytime symptom severity. Symptom intensity was strongly associated with poor quality of life and with the frequency of exacerbations, although it was weakly associated with airflow limitation.

Translation and cultural adaptation of the Sleep Apnea Clinical Score for use in Brazil / Tradução e adaptação cultural do questionário Sleep Apnea Clinical Score para uso no Brasil

ABSTRACT Objective: To translate the Sleep Apnea Clinical Score (SACS) into Brazilian Portuguese and adapt it to the cultural setting, validating it for use as a screening method for polysomnography and as a tool to quantify the risk of obstructive sleep apnea syndrome in individuals in Brazil. Methods: The translation was performed by two professionals, with subsequent synthesis of the translations. From that version, a back-translation was prepared, revised, and compared with the original by a team of experts. As a pre-test, a consensus version was applied in 20 patients randomly selected from among those under treatment at outpatient clinics at the Piquet Carneiro Polyclinic of the State University of Rio de Janeiro, in the city of Rio de Janeiro, to assess their understanding of the questions. In the validation phase, the Brazilian-Portuguese version of the SACS was applied in 86 patients who subsequently underwent polysomnography, regardless of the SACS result. Results: The analyses of the pre-test phase showed that the SACS was easily understood by the patients. In the validation phase, the SACS showed a sensitivity of 45.3% (95% CI: 32.8-58.2%), a specificity of 90.9% (95% CI: 70.8-98.9%), a positive predictive value of 93.5% (95% CI: 79.0-98.2%), a negative predictive value of 36.4% (95% CI: 30.6-42.5%), and an accuracy of 57.0% (95% CI: 45.8-67.6%). Conclusions: The Brazilian-Portuguese version of the SACS can be used in order to assess the risk of obstructive sleep apnea syndrome.

RESUMO Objetivo: Traduzir o questionário Sleep Apnea Clinical Score (SACS) para a língua portuguesa do Brasil, adaptá-lo a nossa cultura e validá-lo para que seja utilizado como método de rastreio para a realização de polissonografia e como ferramenta para quantificar o risco de síndrome da apneia obstrutiva do sono no indivíduo. Métodos: A tradução foi realizada por dois profissionais, com posterior conciliação em uma síntese das traduções. A partir dessa versão, foi elaborada uma tradução reversa, revisada e comparada com o original por uma equipe de especialistas. A versão de consenso foi aplicada em 20 pacientes aleatoriamente selecionados de ambulatórios da Policlínica Piquet Carneiro da Universidade do Estado do Rio de Janeiro, na cidade do Rio de Janeiro (RJ), para avaliar sua compreensão (pré-teste). Posteriormente, a versão brasileira do SACS foi aplicada em 86 pacientes que realizaram polissonografia, independentemente do resultado apresentado no SACS (fase de validação). Resultados: As análises da fase pré-teste demonstraram que o questionário era facilmente compreendido pelos pacientes. Na fase de validação, o questionário demonstrou sensibilidade de 45,3% (IC95%: 32,8-58,2%), especificidade de 90,9% (IC95%: 70,8-98,9%), valor preditivo positivo de 93,5% (IC95%: 79,0-98,2%), valor preditivo negativo de 36,4% (IC95%: 30,6-42,5%) e acurácia de 57,0% (IC95%: 45,8-67,6%). Conclusões: A versão do questionário SACS traduzida para a língua portuguesa do Brasil pode ser utilizada para avaliar o risco de síndrome da apneia obstrutiva do sono.

Symptom variability over the course of the day in patients with stable COPD in Brazil: a real-world observational study / Variabilidade dos sintomas diários de pacientes com DPOC estável no Brasil: um estudo observacional de vida real

ABSTRACT Objective: To analyze symptoms at different times of day in patients with COPD. Methods: This was a multicenter, cross-sectional observational study conducted at eight centers in Brazil. We evaluated morning, daytime, and nighttime symptoms in patients with stable COPD. Results: We included 593 patients under regular treatment, of whom 309 (52.1%) were male and 92 (15.5%) were active smokers. The mean age was 67.7 years, and the mean FEV1 was 49.4% of the predicted value. In comparison with the patients who had mild or moderate symptoms, the 183 (30.8%) with severe symptoms were less physically active (p = 0.002), had greater airflow limitation (p < 0.001), had more outpatient exacerbations (p = 0.002) and more inpatient exacerbations (p = 0.043), as well as scoring worse on specific instruments. The most common morning and nighttime symptoms were dyspnea (in 45.2% and 33.1%, respectively), cough (in 37.5% and 33.3%, respectively), and wheezing (in 24.4% and 27.0%, respectively). The intensity of daytime symptoms correlated strongly with that of morning symptoms (r = 0.65, p < 0.001) and that of nighttime symptoms (r = 0.60, p < 0.001), as well as with the COPD Assessment Test score (r = 0.62; p < 0.001), although it showed only a weak correlation with FEV1 (r = −0.205; p < 0.001). Conclusions: Dyspnea was more common in the morning than at night. Having morning or nighttime symptoms was associated with greater daytime symptom severity. Symptom intensity was strongly associated with poor quality of life and with the frequency of exacerbations, although it was weakly associated with airflow limitation.

RESUMO Objetivo: Analisar os sintomas em diferentes momentos do dia em pacientes com DPOC. Métodos: Estudo observacional multicêntrico de corte transversal em oito centros brasileiros. Foram avaliados os sintomas matinais, diurnos e noturnos em pacientes com DPOC estável. Resultados: Foram incluídos 593 pacientes em tratamento regular, sendo 309 (52,1%) do sexo masculino e 92 (15,5%) fumantes ativos. A média de idade foi de 67,7 anos, e a média de VEF1 foi de 49,4% do valor previsto. Os pacientes com sintomas mais graves (n = 183; 30,8%), em comparação com aqueles com sintomas leves e moderados, apresentaram pior nível de atividade física (p = 0,002), maior limitação ao fluxo aéreo (p < 0,001), exacerbações ambulatoriais (p = 0,002) e hospitalares (p = 0,043) mais frequentemente e piores resultados em instrumentos específicos. Os sintomas matinais e noturnos mais frequentes foram dispneia (em 45,2% e 33,1%, respectivamente), tosse (em 37,5% e 33,3%, respectivamente) e chiado (em 24,4% e 27,0%, respectivamente). Houve forte correlação da intensidade dos sintomas diurnos com sintomas matinais (r = 0,65, p < 0,001), sintomas noturnos (r = 0,60, p < 0,001), bem como com o escore do COPD Assessment Test (r = 0,62; p < 0,001); porém, houve uma correlação fraca com VEF1 (r = −0,205; p < 0,001). Conclusões: A dispneia foi mais frequente no período matinal do que no período noturno. Ter sintomas matinais e/ou noturnos foi associado à pior gravidade dos sintomas diurnos. A intensidade dos sintomas foi fortemente associada a pior qualidade de vida e frequência de exacerbações, mas fracamente associada à limitação ao fluxo aéreo.

Brazilian guidelines for the pharmacological treatment of idiopathic pulmonary fibrosis. Official document of the Brazilian Thoracic Association based on the GRADE methodology / Diretrizes brasileiras para o tratamento farmacológico da fibrose pulmonar idiopática. Documento oficial da Sociedade Brasileira de Pneumologia e Tisiologia baseado na metodologia GRADE

ABSTRACT Idiopathic pulmonary fibrosis (IPF) is a form of chronic interstitial lung disease of unknown cause, which predominantly affects elderly men who are current or former smokers. Even though it is an uncommon disease, it is of great importance because of its severity and poor prognosis. In recent decades, several pharmacological treatment modalities have been investigated for the treatment of this disease, and the classic concepts have therefore been revised. The purpose of these guidelines was to define evidence-based recommendations regarding the use of pharmacological agents in the treatment of IPF in Brazil. We sought to provide guidance on the practical issues faced by clinicians in their daily lives. Patients of interest, Intervention to be studied, Comparison of intervention and Outcome of interest (PICO)-style questions were formulated to address aspects related to the use of corticosteroids, N-acetylcysteine, gastroesophageal reflux medications, endothelin-receptor antagonists, phosphodiesterase-5 inhibitors, pirfenidone, and nintedanib. To formulate the PICO questions, a group of Brazilian specialists working in the area was assembled and an extensive review of the literature on the subject was carried out. Previously published systematic reviews with meta-analyses were analyzed for the strength of the compiled evidence, and, on that basis, recommendations were developed by employing the Grading of Recommendations Assessment, Development and Evaluation approach. The authors believe that the present document represents an important advance to be incorporated in the approach to patients with IPF, aiming mainly to improve its management, and can become an auxiliary tool for defining public policies related to IPF.

RESUMO A fibrose pulmonar idiopática (FPI) é uma forma de pneumopatia intersticial crônica fibrosante de causa desconhecida, que acomete preferencialmente homens idosos, com história atual ou pregressa de tabagismo. Mesmo sendo uma doença incomum, ela assume grande importância devido a sua gravidade e prognóstico reservado. Nas últimas décadas, diversas modalidades terapêuticas farmacológicas foram investigadas para o tratamento dessa doença, de tal modo que conceitos clássicos vêm sendo revisados. O objetivo destas diretrizes foi definir recomendações brasileiras baseadas em evidências em relação ao emprego de agentes farmacológicos no tratamento da FPI. Procurou-se fornecer orientações a questões de ordem prática, enfrentadas pelos clínicos no seu cotidiano. As perguntas PICO (acrônimo baseado em perguntas referentes aos Pacientes de interesse, Intervenção a ser estudada, Comparação da intervenção e Outcome [desfecho] de interesse) abordaram aspectos relativos ao uso de corticosteroides, N-acetilcisteína, tratamento medicamentoso do refluxo gastroesofágico, inibidores dos receptores da endotelina, inibidores da fosfodiesterase-5, pirfenidona e nintedanibe. Para a formulação das perguntas PICO, um grupo de especialistas brasileiros atuantes na área foi reunido, sendo realizada uma extensa revisão bibliográfica sobre o tema. As revisões sistemáticas com meta-análises previamente publicadas foram analisadas quanto à força das evidências compiladas e, a partir daí, foram concebidas recomendações seguindo a metodologia Grading of Recommendations Assessment, Development and Evaluation. Os autores acreditam que o presente documento represente um importante avanço a ser incorporado na abordagem de pacientes com FPI, objetivando principalmente favorecer seu manejo, e pode se tornar uma ferramenta auxiliar na definição de políticas públicas relacionadas à FPI.

Recommendations for the pharmacological treatment of COPD: questions and answers / Recomendações para o tratamento farmacológico da DPOC: perguntas e respostas

ABSTRACT The treatment of COPD has become increasingly effective. Measures that range from behavioral changes, reduction in exposure to risk factors, education about the disease and its course, rehabilitation, oxygen therapy, management of comorbidities, and surgical and pharmacological treatments to end-of-life care allow health professionals to provide a personalized and effective therapy. The pharmacological treatment of COPD is one of the cornerstones of COPD management, and there have been many advances in this area in recent years. Given the greater availability of drugs and therapeutic combinations, it has become increasingly challenging to know the indications for, limitations of, and potential risks and benefits of each treatment modality. In order to critically evaluate recent evidence and systematize the major questions regarding the pharmacological treatment of COPD, 24 specialists from all over Brazil gathered to develop the present recommendations. A visual guide was developed for the classification and treatment of COPD, both of which were adapted to fit the situation in Brazil. Ten questions were selected on the basis of their relevance in clinical practice. They address the classification, definitions, treatment, and evidence available for each drug or drug combination. Each question was answered by two specialists, and then the answers were consolidated in two phases: review and consensus by all participants. The questions answered are practical questions and help select from among the many options the best treatment for each patient and his/her peculiarities.

RESUMO O tratamento da DPOC vem se tornando cada vez mais eficaz. Medidas que envolvem desde mudanças comportamentais, redução de exposições a fatores de risco, educação sobre a doença e seu curso, reabilitação, oxigenoterapia, manejo de comorbidades, tratamentos cirúrgicos e farmacológicos até os cuidados de fim de vida permitem ao profissional oferecer uma terapêutica personalizada e efetiva. O tratamento farmacológico da DPOC constitui um dos principais pilares desse manejo, e muitos avanços têm sido atingidos na área nos últimos anos. Com a maior disponibilidade de medicações e combinações terapêuticas fica cada vez mais desafiador conhecer as indicações, limitações, potenciais riscos e benefícios de cada tratamento. Com o intuito de avaliar criticamente a evidência recente e sistematizar as principais dúvidas referentes ao tratamento farmacológico da DPOC, foram reunidos 24 especialistas de todo o Brasil para elaborar a presente recomendação. Foi elaborado um guia visual para a classificação e tratamento adaptados à nossa realidade. Dez perguntas foram selecionadas pela relevância na prática clínica. Abordam a classificação, definições, tratamento e evidências disponíveis para cada medicação ou combinação. Cada pergunta foi respondida por dois especialistas e depois consolidadas em duas fases: revisão e consenso entre todos os participantes. As questões respondidas são dúvidas práticas e ajudam a selecionar qual o melhor tratamento, entre as muitas opções, para cada paciente com suas particularidades.

Recommendations for the pharmacological treatment of COPD: questions and answers.

The treatment of COPD has become increasingly effective. Measures that range from behavioral changes, reduction in exposure to risk factors, education about the disease and its course, rehabilitation, oxygen therapy, management of comorbidities, and surgical and pharmacological treatments to end-of-life care allow health professionals to provide a personalized and effective therapy. The pharmacological treatment of COPD is one of the cornerstones of COPD management, and there have been many advances in this area in recent years. Given the greater availability of drugs and therapeutic combinations, it has become increasingly challenging to know the indications for, limitations of, and potential risks and benefits of each treatment modality. In order to critically evaluate recent evidence and systematize the major questions regarding the pharmacological treatment of COPD, 24 specialists from all over Brazil gathered to develop the present recommendations. A visual guide was developed for the classification and treatment of COPD, both of which were adapted to fit the situation in Brazil. Ten questions were selected on the basis of their relevance in clinical practice. They address the classification, definitions, treatment, and evidence available for each drug or drug combination. Each question was answered by two specialists, and then the answers were consolidated in two phases: review and consensus by all participants. The questions answered are practical questions and help select from among the many options the best treatment for each patient and his/her peculiarities.

Quando devemos usar CI na DPOC? / When should we use IC in COPD?

Embora as diretrizes para o tratamento da doença pulmonar obstrutiva crônica (dpoc) preconizem o uso de corticoides inalatórios para os casos mais graves e, especialmente, para aqueles que apresentam exacerbações frequentes, verifica-se que o uso destes medicamentos é muito comum inclusive em casos leves e como primeira opção de tratamento. A falta de estudos robustos em pacientes com dpoc e a experiência positiva adquirida no tratamento dos pacientes com asma facilitou a disseminação do uso dos corticoides inalatórios para tratar os pacientes com doença obstrutiva devido ao fumo. Estudos posteriores demonstraram que os corticoides inalados usados isoladamente em pacientes com dpoc não só eram ineficazes, como aumentavam o risco de vários eventos adversos, especialmente a pneumonia. Atualmente, após várias revisões, o documento gold (global initiative for chronic obstructive pulmonary disease) sugere o uso de broncodilatadores combinados para o tratamento de pacientes estáveis. O corticoide inalatório pode ser usado em combinação com um broncodilatador nos pacientes com asma associada, nos que apresentam eosinofilia e naqueles que persistem com exacerbações.

Although the guidelines for the treatment of Chronic Obstructive Pulmonary Disease (COPD) advocate the use of inhaled corticosteroids for more severe cases, and especially for those with frequent exacerbations, the use of these medications is very common even in as the first treatment option. The lack of robust studies in patients with COPD and the positive experience gained in treating patients with asthma facilitated the spread of inhaled corticosteroids in the to treat patients with obstructive disease due to smoking. Subsequent studies have shown that inhaled corticosteroids used alone in COPD patients were not only ineffective, but also increased the risk of some adverse events, especially pneumonia. Currently, after several reviews, the GOLD (Global Initiative for Chronic Obstructive Pulmonary Disease) document suggests the use of combined bronchodilators for the treatment of stable patients. Inhaled corticosteroids may be used in combination with a bronchodilator in patients with associated asthma, those with eosinophilia, and those who persist with exacerbations.

Fenótipos da DPOC: há interesse prático nesta avaliação? / COPD phenotypes: Is it interesting in clinical practice?

A doença pulmonar obstrutiva crônica é causa importante de mortalidade no mundo e está presente em 90-95% dos indivíduos que se expõem ao tabagismo. Há muito tem se discutido a heterogeneidade da doença, inicialmente subdividindo a expressão clínica em dois grandes grupos: "Pink puffer" e "Blow blooter". Recentemente, tem sido descrito diferentes fenótipos da doença subdividindo os pacientes em diversos grupos. Destacam-se como mais prevalentes o exacerbador, a síndrome de sobreposição asma/DPOC e o enfisema com hiperinsuflação. Entretanto, será que devemos, em consulta clínica, estar preocupados em classificar o nosso paciente nestes subgrupos? Essa pergunta é foco de grande discussão, mas à luz dos conhecimentos atuais essa subdivisão parece ser interessante para fins de decisão e escalonamento terapêutico, individualizando o tratamento com o objetivo de maior efetividade terapêutica.

Chronic obstructive pulmonary disease is an important cause of and in 90-95% of cases is associated smoking habit. The heterogeneity of the disease has long been discussed dividing the clinical expression of the disease into two major groups: Pink puffer and Blow blooter. Recently, different phenotypes of the disease have been described, subdividing the patients into several groups. The following stand out: Exacerbator, overlap asthma / COPD syndrome, emphysema with hyperinflation and others. However, is important for us to classify our patients in these subgroups? This question is a focus of great discussion, but the light of current knowledge this subdivision seems to be interesting to decide about which drug we will choose to achieve individual effectiveness.

Rituximab for the therapy of systemic sclerosis: a series of 10 cases in a single center.

Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Although cyclophosphamide is effective for severe and refractory cases, there is demand for new treatments. The biological treatment with B-cell depletion with rituximab (RTX) has demonstrated efficacy for this demand in open-label studies. OBJECTIVE: This study was conducted with the aim to retrospectively evaluate all patients who used RTX for the treatment of SSc in our center. PATIENTS AND METHODS: We retrospectively evaluated medical records of all patients with SSc who used RTX to treat this disease from January 2009 to January 2015. Systemic, cutaneous, and pulmonary involvement data and laboratory results before and six months after the first infusion of RTX were collected. RESULTS: Ten patients received treatment during the study period and were included in this series. All patients had a diffuse form of the disease. Five patients suffered from an early (duration of disease shorter or equal to four years), rapidly progressive disease, and another five received RTX at late stages of the disease. In both groups of patients, stabilization of the pulmonary picture was observed, with a fall in the skin score in those patients with early forms of the disease. DISCUSSION: Similar to findings in previous studies, RTX was effective in treating early and rapidly progressive forms of SSc. We also found that patients with long-term illness may benefit from the treatment.

Rituximab for the therapy of systemic sclerosis: a series of 10 cases in a single center / Tratamento da esclerose sistêmica com rituximabe: uma série de 10 casos em centro único

Abstract Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Although cyclophosphamide is effective for severe and refractory cases, there is demand for new treatments. The biological treatment with B-cell depletion with rituximab (RTX) has demonstrated efficacy for this demand in open-label studies. Objective This study was conducted with the aim to retrospectively evaluate all patients who used RTX for the treatment of SSc in our center. Patients and methods We retrospectively evaluated medical records of all patients with SSc who used RTX to treat this disease from January 2009 to January 2015. Systemic, cutaneous, and pulmonary involvement data and laboratory results before and six months after the first infusion of RTX were collected. Results Ten patients received treatment during the study period and were included in this series. All patients had a diffuse form of the disease. Five patients suffered from an early (duration of disease shorter or equal to four years), rapidly progressive disease, and another five received RTX at late stages of the disease. In both groups of patients, stabilization of the pulmonary picture was observed, with a fall in the skin score in those patients with early forms of the disease. Discussion Similar to findings in previous studies, RTX was effective in treating early and rapidly progressive forms of SSc. We also found that patients with long-term illness may benefit from the treatment.

Resumo A esclerose sistêmica (ES) é uma doença autoimune crônica de alta morbimortalidade. Ainda que a ciclofosfamida seja eficaz, para casos graves e refratários há demanda para novos tratamentos. A terapia biológica com depleção de células B com rituximabe (RTX) demonstrou eficácia para tal demanda em estudos abertos. Objetivo Avaliar retrospectivamente todos os pacientes que fizeram uso do RTX para tratamento de ES em nosso centro. Pacientes e métodos Foram avaliados retrospectivamente todos os prontuários de pacientes com ES que fizeram uso de RTX para tratamento da ES de janeiro de 2009 a janeiro de 2015. Dados de acometimento sistêmico, cutâneo, pulmonar e laboratoriais antes e seis meses após a primeira infusão de RTX foram coletados. Resultados Dez pacientes receberam o tratamento no período de estudo e foram incluídos na presente série de casos. Todos os pacientes tinham a forma difusa da doença. Cinco pacientes tinham formas iniciais (tempo de doença menor ou igual a quatro anos) e rapidamente progressiva da doença e cinco receberam o RTX em fases tardias da doença. Houve estabilização do quadro pulmonar em ambos os grupos de pacientes e redução no escore cutâneo nos pacientes com formas iniciais da doença. Discussão Similar ao encontrado em estudos prévios, o RTX foi eficaz no tratamento de formas iniciais e rapidamente progressivas da ES. Verificamos também benefício em pacientes com longa duração da doença.