Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

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Histological features and survival in idiopathic pulmonary fibrosis

Achados histológicos e sobrevida na fibrose pulmonar idiopática

Ester Nei Aparecida Martins Coletta, Carlos Alberto de Castro Pereira, Rimarcs Gomes Ferreira, Adalberto Sperb Rubin, Lucimara Sonja Villela, Tatiana Malheiros, João Norberto Stávale

J Bras Pneumol.2003;29(6):371-378

Abstract PDF PT

Background: Idiopathic pulmonary fibrosis was recently redefined as usual interstitial pneumonia of unknown etiology. Consequently, the prognostic value of histological findings needs to be reassessed. Objective: To correlate clinical, functional and histological findings with survival in patients with idiopathic pulmonary fibrosis. Method: Patients (n = 51; mean age: 66 ± 8 years; gender: 21 females/30 males) were evaluated. Of the 51, 26 were smokers or ex-smokers. Duration of symptoms, forced vital capacity and smoking habits were recorded. All patients presented usual interstitial pneumonia verified through histology. Degree of honeycombing, established fibrosis, desquamation, cellularity, myointimal thickening of blood vessels and number of fibroblastic foci were graded according to the semiquantitative method. Results: Median duration of symptoms was 12 months and initial forced vital capacity was 72 ± 21%. Cox multivariate analysis revealed that survival correlated inversely and significantly (p < 0.05) with duration of symptoms and fibroblastic foci score, as well as with myointimal thickening of blood vessels. Limited numbers of fibroblastic foci, as well as myointimal thickening involving less than 50% of blood vessels, were predictive of greater survival. No correlation with survival was found for gender, age, forced vital capacity, inflammation or degree of cellularity. Conclusion: Semiquantitative analysis of lung biopsies yields relevant prognostic information regarding patients with usual interstitial pneumonia.

 


Keywords: Pulmonary fibrosis. Lung diseases, interstitial. Survival analysis.

 


Highlights of the Brazilian Thoracic Association Guidelines for Interstitial Lung Diseases

Destaques das Diretrizes de Doenças Pulmonares Intersticiais da Sociedade Brasileira de Pneumologia e Tisiologia

Bruno Guedes Baldi, Carlos Alberto de Castro Pereira, Adalberto Sperb Rubin, Alfredo Nicodemos da Cruz Santana, André Nathan Costa, Carlos Roberto Ribeiro Carvalho, Eduardo Algranti, Eduardo Mello de Capitani, Eduardo Pamplona Bethlem, Ester Nei Aparecida Martins Coletta, Jaquelina Sonoe Ota Arakaki, José Antônio Baddini Martinez, Jozélio Freire de Carvalho, Leila John Marques Steidle, Marcelo Jorge Jacó Rocha, Mariana Silva Lima, Maria Raquel Soares, Marlova Luzzi Caramori, Miguel Abidon Aidé, Rimarcs Gomes Ferreira, Ronaldo Adib Kairalla, Rudolf Krawczenko Feitoza de Oliveira, Sérgio Jezler, Sílvia Carla Sousa Rodrigues, Suzana Pinheiro Pimenta

J Bras Pneumol.2012;38(3):282-291

Abstract PDF PT PDF EN Portuguese Text

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

 


Keywords: Lung diseases, interstitial; Guidelines as topic; Brazil.

 


Effects of salbutamol delivered by dry-powder inhaler on methacholine-induced bronchoconstriction

Efeito do salbutamol liberado através de inalador de pó seco sobre o broncoespasmo induzido por metacolina

Adalberto Sperb Rubin, Liliana G Pelegrin,Christiano Perin, Maurício Roux Leite, Luiz Carlos Corrêa da Silva

J Bras Pneumol.2004;30(3):195-200

Abstract PDF PT

Background: Short-acting b2 agonists delivered by metered-dose inhaler (MDIs) are the drugs usually used for the reversal of methacholine-induced bronchoconstriction. The b2 agonists that are delivered by dry-powder inhaler (DPI) can be an efficacious option. Objective: To evaluate the effectiveness and speed of action of salbutamol delivered by DPI (Pulvinal; Butoventâ), in comparison to salbutamol delivered by MDI, in reversing methacholine-induced bronchoconstriction. Method: Sixty successive methacholine-induced bronchoconstriction patients who presented a decrease of at least 20% in forced expiratory volume (FEV1) were evaluated prospectively. Of these 60 patients, we randomized 30 (first group) to receive 200 mcg of salbutamol by MDI and 30 (second group) to receive 200 mcg of salbutamol by DPI (Pulvinal). Both drugs were administered with the objective of reversing bronchoconstriction during the final phase of a bronchoprovocation test. The FEV1 values obtained at 1 and 5 minutes after bronchodilator administration were evaluated. Results: The groups were comparable in gender distribution, age, weight, dose level provoking a 20% drop in FEV1 (first group: 1.3 mg; second group: 1.19 mg; p = 0.79) and post-methacholine FEV1 (first group: 2.03 l; second group: 1.99 l; p = 0.87), with no statistically significant differences between the two groups. In the first group (MDI), the mean increase in FEV1 was 16.2% (at 1 minute) and 22.2% (at 5 minutes), and in the second group (DPI) it was 17% (at 1 minute) and 23.6% (at 5 minutes). There was no statistically significant difference between the groups (p = 0.8). Conclusion: The b2-agonists delivered by DPI (Pulvinal) present the same bronchodilator efficacy and speed of action as do those delivered by the more traditional MDI method.

 


Keywords: Asthma. Bronchodilator agents/administration & dosage. Albuterol/administration & dosage. Methacholine chloride/administration & dosage. Respiratory therapy/methods. Administration, inhalation/methods. Prospective studies.

 


Efficacy of inhaled formoterol in reversing bronchoconstriction

Eficácia do formoterol na reversão imediata do broncoespasmo

Adalberto Sperb Rubin, Christiano Perin, Liliana Pelegrin, Juliana Cardozo Fernandes, Luiz Carlos Corrêa da Silva

J Bras Pneumol.2006;32(3):202-206

Abstract PDF PT PDF EN Portuguese Text

Objective: To evaluate the effectiveness and onset of action of formoterol delivered by dry-powder inhaler in reversing methacholine-induced bronchoconstriction. Methods: Patients presenting a drop in forced expiratory volume in one second > 20% after methacholine inhalation were included. A total of 84 patients were evaluated. All of the participating patients presented respiratory symptoms of unknown origin, which were being investigated. The patients were randomized to receive 200 µg of spray fenoterol (n = 41) or 12 µg of dry-powder inhaler formoterol (n = 43), both administered in order to achieve immediate reversal of methacholine-induced bronchoconstriction. We evaluated the decrease in forced expiratory volume in one second (in relation to the baseline value) after methacholine challenge and the dose of methacholine required to provoke a drop of 20% in forced expiratory volume in one second, as well as the increase in forced expiratory volume in one second (in relation to the baseline value) at five and ten minutes after bronchodilator use. Results: There were no significant differences related to gender, age, weight, height or dose of methacholine required to provoke a drop of 20% in forced expiratory volume in one second. Nor were there any significant differences in terms of baseline or post-methacholine forced expiratory volume in one second. In the fenoterol group, the mean postbronchodilator increase in forced expiratory volume in one second increase was 34% (at five minutes) and 50.1% (at ten minutes), compared with 46.5% (at five minutes) and 53.2% (at ten minutes) in the formoterol group. Conclusion: The bronchodilator effect of formoterol at five and ten minutes after methacholine-induced bronchoconstriction was similar to that of fenoterol. Despite being a long-acting bronchodilator, formoterol also has a rapid onset of action, which suggests that it could be employed as a relief medication in cases of bronchoconstriction occurring during asthma attacks.

 


Keywords: Asthma; Formoterol; Fenoterol; Inhalation therapy; Bronchodilation, Methacoline

 


Prognostic factors in idiopathic pulmonary fibrosis

Fatores prognósticos em fibrose pulmonar idiopática

Adalberto Sperb Rubin, José da Silva Moreira, Nelson da Silva Porto, Klaus Loureiro Irion, Rafael Franco Moreira, Bruno Ssheidt

J Bras Pneumol.2000;26(5):227-234

Abstract PDF PT

In order to evaluate which prognostic factors were significant to the survival of patients with idiopathic pulmonary fibrosis (IPF), 121 histologically confirmed cases of the disease were studied at the Pereira Filho Hospital from 1970 to 1996. All patients were submitted to a standard thorax X-ray and spirometry and answered a standardized questionnaire when admitted to hospital. They also underwent diffusion tests (34 cases), total lung capacity (28), blood gas analysis (106), bronchoalveolar lavage (39), rheumatoid analyses (45), and thoracic CT (24). For further analysis, the patients were classified into two groups: group A (2-year survival) with 55 patients, and group B (more than 5-year survival) with 24 patients; these features were also analyzed according to their significance to survival. Age, increased dyspnea index, long symptomatic period, FVC, DCO, PaO2 and SaO2 reduction, honeycombing intensity, and greater profusion of the reticular pattern on HRCT were considered indicative of worse prognosis. A reduced FEV1 and TLC were also associated with shorter survival. The use of those criteria which had shown statistical significance when evaluated together may determine a more accurate prognostic evaluation of IPF patients resulting in social and therapeutic benefits to patient management.

 


Keywords: Pulmonary fibrosis. Interstitial lung diseases. Prognosis. Prospective studies. Survival analysis.

 


Pulmonary idiopathic fibrosis: clinical findings and survival in 132 histologically-proven patients

Fibrose pulmonar idiopática: características clínicas e sobrevida em 132 pacientes com comprovação histológica

Adalberto Sperb Rubin, José da Silva Moreira, Nelson da Silva Porto, Klaus Loureiro Irion, Rafael Franco Moreira, Bruno Scheidt

J Bras Pneumol.2000;26(2):61-68

Abstract PDF PT

In order to evaluate the clinical findings and survival of pulmonary idiopathic fibrosis patients, 132 cases with histologically-proven biopsy were studied, coming from Pavilhão Pereira Filho Hospital, from 1970 to 1996. The diagnosis was made in 120 patients by open lung biopsy and in 12 cases by transbronchial lung biopsy. The average age was 56 years; 78 were male and only 6 were black. Smoking was observed in 61 cases. Mean duration of symptoms before diagnosis was 22.7 months. Digital clubbing was present in 75 patients and teleinspiratory crackles in 100. Dyspnea was observed in all but two patients and cough was present in 89 cases. Lung function test values were: FVC, 62%; FEV1, 70%; DLCO, 43.4%; TLC, 76.7%; PaO2, 67.3 mmHg; PaCO2, 39.1 mmHg and SaO2, 92.3%. Bronchoalveolar cellularity values were: macrophages, 83.8%; neutrophils, 9.1%; lymphocytes, 6.1% and eosinophils, 0.6%. In X-ray, honeycombing was present in 79 cases, reduced total lung capacity in 107 and intrathoracic tracheal widening in 50. In CT, the mean reticular pattern profusion was 42.3% and the mean granular pattern profusion was 43.6%. The usual histologic pattern was found in 128 cases, and the descamative pattern in only 4. Information about survival was found in 121 cases until December 1997. The mean survival rate of all patients was 28 months and for dead patients was 24 months. Patient characteristics in this study were associated with advanced stage of disease, which was confirmed by small survival rates of those cases. The strong predominance of usual pattern and better patient selection may have contributed to these results.

 


Keywords: Pulmonary fibrosis, interstitial lung disease, clinical symptoms; Survival analysis.

 


Functional improvement in patients with idiopathic pulmonary fibrosis undergoing single lung transplantation

Melhora funcional em portadores de fibrose pulmonar idiopática submetidos a transplante pulmonar unilateral

Adalberto Sperb Rubin1,2, Douglas Zaione Nascimento1, Letícia Sanchez1, Guilherme Watte2, Arthur Rodrigo Ronconi Holand1, Derrick Alexandre Fassbind1, José Jesus Camargo1,2

J Bras Pneumol.2015;41(4):299-304

Abstract PDF PT PDF EN Portuguese Text

Objective: To evaluate the changes in lung function in the first year after single lung transplantation in patients with idiopathic pulmonary fibrosis (IPF). Methods: We retrospectively evaluated patients with IPF who underwent single lung transplantation between January of 2006 and December of 2012, reviewing the changes in the lung function occurring during the first year after the procedure. Results: Of the 218 patients undergoing lung transplantation during the study period, 79 (36.2%) had IPF. Of those 79 patients, 24 (30%) died, and 11 (14%) did not undergo spirometry at the end of the first year. Of the 44 patients included in the study, 29 (66%) were men. The mean age of the patients was 57 years. Before transplantation, mean FVC, FEV1, and FEV1/FVC ratio were 1.78 L (50% of predicted), 1.48 L (52% of predicted), and 83%, respectively. In the first month after transplantation, there was a mean increase of 12% in FVC (400 mL) and FEV1 (350 mL). In the third month after transplantation, there were additional increases, of 5% (170 mL) in FVC and 1% (50 mL) in FEV1. At the end of the first year, the functional improvement persisted, with a mean gain of 19% (620 mL) in FVC and 16% (430 mL) in FEV1. Conclusions: Single lung transplantation in IPF patients who survive for at least one year provides significant and progressive benefits in lung function during the first year. This procedure is an important therapeutic alternative in the management of IPF.

 


Keywords: Pulmonary fibrosis; Respiratory function tests; Lung transplantation.

 


Mortality due to idiopathic pulmonary fibrosis in the State of Rio Grande do Sul (Brazil)

Mortalidade por fibrose pulmonar idiopática no Rio Grande do Sul

Fabrício Picolli Fortuna, Christiano Perin, Letícia Cunha, Adalberto Sperb Rubin

J Bras Pneumol.2003;29(3):121-124

Abstract PDF PT

Epidemiologic data on idiopathic pulmonary fibrosis are relatively scarce, and its real incidence and prevalence are unknown. Recent studies suggest that idiopathic pulmonary fibrosis mortality is rising in developed countries. Objectives: To describe idiopathic pulmonary fibrosis mortality in the State of Rio Grande do Sul (RS), Brazil, from 1970 to 2000, analyzing its trend and comparing it with that from other countries. Methods: Prevalence study, using data from the Brazilian Institute of Geography and Statistics (IBGE), analyzing death certificates in which idiopathic pulmonary fibrosis was stated as ultimate cause of death. Results: Annual mortality adjusted to population was 0.22/100,000 persons in the 1970's, 0.3/100,000 persons in the 1980's, and 0.48/100,000 persons in the 1990's. Total mortality rised 36% from 1970 to 1980, and 70% from 1980 to 1990. Mortality adjusted to population rised 36% and 60% during the same periods. The rise in both total and adjusted mortality from IPF was statistically significant (p < 0.05). The mean mortality rate per 100,000 persons during 1996 to 1998, however, was 0.683, corresponding to a rise of 70% when compared to the previous three-year period, which was 0.4 (p = 0.0002), probably reflecting encoding practices. Conclusion: There was a significant increase in IPF mortality in RS from 1970 to 2000, in part due to changes in coding practices. This increase is in conformity with observations in other countries, althought mortality rates in RS are considerably lower.

 


Keywords: Pulmonary fibrosis. Mortality.

 


Multiple pulmonary metastases of benign meningioma

Múltiplas metástases pulmonares de meningioma benigno

Adalberto Sperb Rubin, Liliana Gomes Pellegrin, Nelson da Silva Porto, Geraldo Geyer.

J Bras Pneumol.2005;31(2):177-180

Abstract PDF PT PDF EN Portuguese Text

Meningiomas account for approximately one-sixth of all primary neoplasms of the central nervous system and rarely present extracranial metastases. A finding of multiple metastases is rare, as is the presence of respiratory symptoms. Herein, we report the case of a 67-year-old female patient presenting subacute onset of respiratory symptoms. The patient had undergone resection of a benign intracranial meningioma six months prior, but had no history of lung disease. Upon examination, multiple pulmonary metastases, originating from the primary tumor, were observed.

 


Keywords: Key words: Meningioma. Neoplasm Metastasis. Lung Neoplasms..

 


Clinical and radiographic spectrum of organizing pneumonia: retrospective analysis of 38 cases

O espectro clínico e radiológico da pneumonia em organização: análise retrospectiva de 38 casos

Fabrício Piccoli Fortuna, Cristiano Perin, Juliano de Bortoli, Geraldo Resin Geyer, Nelson da Silva Porto, Adalberto Sperb Rubin

J Bras Pneumol.2002;28(6):317-323

Abstract PDF PT

Organizing pneumonia, whether or not accompanied by bronchiolitis obliterans, is a specific anatomicopathological condition of the lungs that can present in a variety of clinical and radiographic ways. It can be either idiopathic or secondary to a number of diseases, including infection and drugs. Objectives: To describe the clinical manifestations, radiographic and spirometric data seen in patients with organizing pneumonia. Methods: Retrospective analysis of patients with a diagnosis of organizing pneumonia. Results: 38 patients were included in the analysis. Fourteen also had clinical conditions related to organizing pneumonia, and they presented more frequently with diffuse pulmonary infiltrates (three of four cases) and associated bronchiolitis obliterans (57% vs. 20%, p = 0,05). Of the 13 patients with bronchiolitis obliterans, only one was asymptomatic and two had localized lesions (15%). Of the eight asymptomatic patients with localized lesions, no one had associated bronchiolitis obliterans, and the radiographic appearance often resembled bronchial carcinoma. Spirometry was generally of little value to diagnosis, probably because of the high prevalence of smoking in the sample. Conclusion: The clinical and radiographic presentation of organizing pneumonia is variable. Factors that suggest the absence of coexisting bronchiolitis obliterans are the absence of symptoms and localized radiographic lesions, and this form of the disease has to be more often differentiated from bronchial carcinoma in clinical practice.

 



Measuring forced expiratory volume in one second alone is not an accurate method of assessing response to bronchodilators in chronic obstructive pulmonary disease

O volume expiratório forçado no primeiro segundo não é suficiente para avaliar resposta broncodilatadora em doença pulmonar obstrutiva crônica

Felícia de Moraes Branco Tavares, Luiz Carlos Corrêa da Silva, Adalberto Sperb Rubin

J Bras Pneumol.2005;31(5):407-414

Abstract PDF PT PDF EN Portuguese Text

Objective: To assess the frequency of variation in forced expiratory volume in one second after bronchodilator use in a sample of patients with chronic obstructive pulmonary disease, correlating such variation with clinical and demographic variables and evaluating the frequency of response presented in forced vital capacity, slow vital capacity, inspiratory capacity, residual volume, airway resistance and specific airway conductance. Methods: A total of 64 patients with chronic obstructive pulmonary disease were submitted to whole body plethysmography, and reversibility of bronchoconstriction after the administration 400 µg of fenoterol was quantified. Results: A response in forced expiratory volume in one second was observed in 31% of the patients. Excluding patients presenting a response in forced expiratory volume in one second, 5% presented responses in 5 of the other 6 parameters, 10% presented responses in 4 parameters, 17.5% in 3 parameters, 27.5% in 2 parameters, and 25% in only 1 parameter. Conclusion: When included in the evaluation of bronchodilator response together with forced expiratory volume in one second, static lung volumes, airway resistance and airway conductance allowed a broader evaluation of those patients presenting a functional pharmacodynamic response. These results are in accordance with the observation that bronchodilator use provides clinical improvement and relief of dyspnea to many patients with chronic obstructive pulmonary disease, even to those in whom such treatment leads to no improvement in forced expiratory volume in one second.

 


Keywords: Pulmonary disease, chronic obstructive/drug therapy; Bronchodilator agents/therapeutic use; Airway resistance/drug effects; Respiratory function tests; Vital capacity; Forced expiratory volume

 


Chronic eosinophilic pneumonia

Pneumonia eosinofílica crônica

Alessandra Isabel Zille, Christiano Perin, Geraldo Resin Geyer, Jorge Lima Hetzel, Adalberto Sperb Rubin

J Bras Pneumol.2002;28(5):281-284

Abstract PDF PT

Chronic eosinophilic pneumonia is a rare disease of unknown cause characterized by eosinophilic alveolar and interstitial infiltration. The authors describe the case of a 49-year-old caucasian woman, presenting dyspnea on minimum effort, with insidious beginning and progressive course in the last six months. The main findings were serum eosinophilia and in the sputum, chest radiographs showing multifocal infiltrations of irregular distribution in both lungs and a restrictive functional impairment. The patient was submitted to an open lung biopsy, which demonstrated a chronic eosinophilic pneumonia. There was a dramatic clinical, radiological, and functional response after corticosteroid therapy.

 



Predicting reduced TLC in patients with low FVC and a normal or elevated FEV1/FVC ratio

Predizendo redução da CPT em pacientes com CVF reduzida e relação VEF1/CVF normal ou elevada

Luiz Carlos D'Aquino, Sílvia Carla Sousa Rodrigues, João Adriano de Barros, Adalberto Sperb Rubin, Nelson Augusto Rosário Filho, Carlos Alberto de Castro Pereira

J Bras Pneumol.2010;36(4):-

Abstract PDF PT PDF EN Portuguese Text

Objective: To use clinical and spirometry findings in order to distinguish between the restrictive and nonspecific patterns of pulmonary function test results in patients with low FVC and a normal or elevated FEV1/FVC ratio. Methods: We analyzed the pulmonary function test results of 211 adult patients submitted to spirometry and lung volume measurements. We used the clinical diagnosis at the time spirometry was ordered, together with various functional data, in order to distinguish between patients presenting with a "true" restrictive pattern (reduced TLC) and those presenting with a nonspecific pattern (normal TLC). Results: In the study sample, TLC was reduced in 144 cases and was within the normal range in 67. The most common causes of a nonspecific pattern were obstructive disorders, congestive heart failure, obesity, bronchiolitis, interstitial diseases, and neuromuscular disorders. In patients given a working diagnosis of pulmonary fibrosis, pleural disease, or chest wall disease, the positive predictive value (PPV) for restriction was ≥ 90%. In males, an FVC ≤ 60% of predicted had a PPV for restriction of 98.8%. In females, the restrictive pattern was found in 84.4% of those with an FVC ≤ 50% of predicted. A difference of ≥ 0% between the FEV1% and the FVC% had a PPV for restriction of 89.5%. After performing logistic regression, we developed a point scale for predicting the restrictive pattern. Conclusions: In many patients with reduced FEV1, reduced FVC, and a normal FEV1/FVC ratio, the restrictive pattern can be identified with confidence through the use of an algorithm that takes the clinical diagnosis and certain spirometry measurements into account.

 


Keywords: Spirometry; Airway resistance; Respiratory function tests; Vital capacity.

 


Prevalence of gastroesophageal reflux disease in patients with idiopathic pulmonary fibrosis

Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática

Cristiane Dupont Bandeira, Adalberto Sperb Rubin, Paulo Francisco Guerreiro Cardoso, José da Silva Moreira, Mirna da Mota Machado

J Bras Pneumol.2009;35(12):1182-1189

Abstract PDF PT PDF EN Portuguese Text

Objective: To determine the prevalence of gastroesophageal reflux disease (GERD) and to evaluate its clinical presentation, as well as the esophageal function profile in patients with idiopathic pulmonary fibrosis (IPF). Methods: In this prospective study, 28 consecutive patients with IPF underwent stationary esophageal manometry, 24-h esophageal pH-metry and pulmonary function tests. All patients also completed a symptom and quality of life in GERD questionnaire. Results: In the study sample, the prevalence of GERD was 35.7%. The patients were then divided into two groups: GERD+ (abnormal pH-metry; n = 10) and GERD− (normal pH-metry; n = 18). In the GERD+ group, 77.7% of the patients presented at least one typical GERD symptom. The pH-metry results showed that 8 (80%) of the GERD+ group patients had abnormal supine reflux, and that the reflux was exclusively in the supine position in 5 (50%). In the GERD+ and GERD− groups, respectively, 5 (50.0%) and 7 (38.8%) of the patients presented a hypotensive lower esophageal sphincter, 7 (70.0%) and 10 (55.5%), respectively, presenting lower esophageal dysmotility. There were no significant differences between the groups regarding demographic characteristics, pulmonary function, clinical presentation or manometric findings. Conclusions: The prevalence of GERD in the patients with IPF was high. However, the clinical and functional characteristics did not differ between the patients with GERD and those without.

 


Keywords: Pulmonary fibrosis; Gastroesophageal reflux; Prevalence; Manometry; Esophageal pH monitoring.

 


Immediate bronchodilator response to formoterol in poorly reversible chronic obstructive pulmonary disease

Resposta broncodilatadora imediata ao formoterol em doença pulmonar obstrutiva crônica com pouca reversibilidade

Adalberto Sperb Rubin, Fábio José Fabrício de Barros Souza, Jorge Lima Hetzel, José da Silva Moreira

J Bras Pneumol.2008;34(6):373-379

Abstract PDF PT PDF EN Portuguese Text

Objective: To evaluate, using pulmonary function tests, the effectiveness of formoterol as a bronchodilator at 30 min after its administration in patients with poorly reversible COPD. Methods: A prospective study including 40 COPD patients not responding to the short-acting bronchodilator used in the spirometric test-variation of less than 200 mL and less than 7% of predicted in forced expiratory volume in one second (FEV1). All patients were classified as having stage II, III, or IV COPD (Brazilian Thoracic Society/Global Initiative for Chronic Obstructive Lung Disease) and presented FEV1 ≤ 70% of predicted value. The patients were randomized into two groups of 20, with similar clinical characteristics, receiving, via a dry powder inhaler, either formoterol or a placebo. The pulmonary function testing (plethysmography) was repeated at 30 min after formoterol or placebo administration. Results: In the formoterol group, the mean values obtained for FEV1, inspiratory capacity, and forced vital capacity were significantly greater than those obtained in the placebo group (p = 0.00065, p = 0.05, and p = 0.017, respectively), whereas that obtained for airway resistance was significantly lower (p = 0.010). Less pronounced differences were observed for residual volume, vital capacity and specific airway conductance, which were lower, higher and higher, respectively, in the formoterol group. Conclusions: In COPD patients not responding to the short-acting bronchodilator used in the spirometric test, formoterol promoted significant improvement in lung function at 30 min after of administration. Further studies are required to confirm whether formoterol can also be used as a medication for immediate relief of symptoms in COPD.

 


Keywords: Chronic obstructive pulmonary disease; Respiratory function tests; Bronchodilator agents.

 


Magnetic resonance of the lung: a step forward in the study of lung disease

Ressonância magnética de pulmão: um novo passo no estudo das doenças pulmonares

Bruno Hochhegger, Edson Marchiori, Klaus Irion, Arthur Soares Souza Junior, Jackson Volkart, Adalberto Sperb Rubin

J Bras Pneumol.2012;38(1):105-115

Abstract PDF PT PDF EN Portuguese Text

Magnetic resonance imaging (MRI) of the lung has progressed tremendously in recent years. Because of improvements in speed and image quality, MRI is now ready for routine clinical use. The main advantage of MRI of the lung is its unique combination of structural and functional assessment in a single imaging session. We review the three major clinical indications for MRI of the lung: staging of lung tumors; evaluation of pulmonary vascular disease; and investigation of pulmonary abnormalities in patients who should not be exposed to radiation.

 


Keywords: Magnetic resonance imaging; Lung; Lung diseases; Carcinoma, non-small-cell lung; Lung diseases, interstitial; Pneumonia.

 


Safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis in Brazil

Segurança e tolerabilidade de Nintedanibe em pacientes com fibrose pulmonar idiopática no Brasil

Carlos Alberto de Castro Pereira1,a, José Antonio Baddini-Martinez2,b, Bruno Guedes Baldi3,c, Sérgio Fernandes de Oliveira Jezler4,d, Adalberto Sperb Rubin5,e, Rogerio Lopes Rufino Alves6,f, Gilmar Alves Zonzin7,g, Manuel Quaresma8,h, Matthias Trampisch9,i, Marcelo Fouad Rabahi10,j

J Bras Pneumol.2019;45(5):e20180414-e20180414

Abstract PDF PT PDF EN Portuguese Text

Objective: Clinical trials have shown that nintedanib 150 mg twice daily (bid) reduces disease progression in patients with idiopathic pulmonary fibrosis (IPF), with an adverse event profile that is manageable for most patients. Prior to the approval of nintedanib as a treatment for IPF in Brazil, an expanded access program (EAP) was initiated to provide early access to treatment and to evaluate the safety and tolerability of nintedanib in this patient population. Methods: Patients with a diagnosis of IPF within the previous five years, forced vital capacity (FVC) ≥ 50% predicted and diffusing capacity of the lungs for carbon monoxide (DLco) 30% to 79% predicted were eligible to participate in the EAP. Patients received nintedanib 150 mg bid open-label. Safety assessments included adverse events leading to permanent discontinuation of nintedanib and serious adverse events. Results: The EAP involved 57 patients at eight centers. Most patients were male (77.2%) and white (87.7%). At baseline, mean (SD) age was 70.7 (7.5) years and FVC was 70.7 (12.5) % predicted. Mean (SD) exposure to nintedanib was 14.4 (6.2) months; maximum exposure was 22.0 months. The most frequently reported adverse events considered by the investigator to be related to nintedanib treatment were diarrhea (45 patients, 78.9%) and nausea (25 patients, 43.9%). Adverse events led to permanent discontinuation of nintedanib in 16 patients (28.1%). Sixteen patients (28.1%) had a serious adverse event. Conclusion: In the Brazilian EAP, nintedanib had an acceptable safety and tolerability profile in patients with IPF, consistent with data from clinical trials.

 


Keywords: Drug tolerance; Expanded access program; Interstitial lung disease; Tyrosine kinase inhibitor.

 


Bronchial thermoplasty in asthma

Termoplastia brônquica em asma

Adalberto Sperb Rubin, Paulo Francisco Guerreiro Cardoso

J Bras Pneumol.2010;36(4):-

Abstract PDF PT PDF EN Portuguese Text

Currently available treatments for asthma provide satisfactory control of the disease in most cases. However, a significant number of patients do not respond to such treatments (i.e., do not achieve effective symptom relief). One novel approach to treating asthma is bronchial thermoplasty, in which the airway smooth muscle is specifically and directly treated. This procedure delivers radiofrequency energy to the airways in order to reduce smooth muscle-mediated bronchoconstriction. In this article, we present the thermoplasty technique, summarizing the results of the major randomized clinical trials of the procedure, as well as discussing its mechanisms of action and potential adverse effects. We also propose strategies for the future clinical use of this new treatment.

 


Keywords: Asthma/prevention & control; Asthma/therapy; Bronchoscopy.

 


Bronchial thermoplasty: report on the first endoscopic treatment for asthma in Latin America*

Termoplastia brônquica: relato do primeiro tratamento endoscópico de asma na América Latina

Adalberto Sperb Rubin, Paulo Francisco Guerreiro Cardoso

J Bras Pneumol.2008;34(1):59-62

Abstract PDF PT PDF EN Portuguese Text

Bronchial thermoplasty is a new bronchoscopic procedure that delivers radiofrequency energy to the airway and potentially reduces the smooth muscle-mediated bronchoconstriction. We report the case of a 48-year-old man with persistent moderate asthma submitted to bronchial thermoplasty. The treatment increased the forced expiratory volume in one second, increased the number of symptom-free days, reduced the use of relief medications, and improved the Juniper Asthma Quality of Life Scale score. In this patient, bronchial thermoplasty was well tolerated and safe. This was the first bronchial thermoplasty performed in Latin America. At 12 months after the procedure, the results were encouraging in terms of its potential benefits in patients with difficult-to-control asthma.

 


Keywords: Asthma/treatment; Respiratory function tests; Case reports [publication type].

 


 

 


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