que a posição obtida e o valor do indicador, a volta do JBP ao relatório anual da base de dados g... more que a posição obtida e o valor do indicador, a volta do JBP ao relatório anual da base de dados geradora do Fator de Impacto demonstra que o caminho trilhado ao longo dos últimos anos sempre foi pautado pela divulgação da ciência de qualidade na área respiratória. Conforme mencionado, o Fator de Impacto do JBP, pelo mecanismo através do qual é feito seu cálculo, ainda espelhará o efeito da penalidade sofrida ao longo de pelo menos os próximos dois anos; ainda assim, o retorno do JBP consolida sua posição dentre as revistas de relevância para a divulgação da área de medicina respiratória no mundo.
1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung... more 1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung transfer ratio: physiological implications. J Physiol 2007; 582: 767-775. 2 Hughes JM, van der Lee I. The TL,NO/TL,CO ratio in pulmonary function test interpretation. Eur Respir J 2013; 41: 453-461. 3 Guenard H, Varene N, Vaida P. Determination of lung capillary blood volume and membrane diffusing capacity in man by the measurements of NO and CO transfer. Respir Physiol 1987; 70: 113-120. 4 Roughton FJ, Forster RE. Relative importance of diffusion and chemical reaction rates in determining rate of exchange of gases in the human lung, with special reference to true diffusing capacity of pulmonary membrane and volume of blood in the lung capillaries. J Appl Physiol 1957; 11: 290-302. 5 Forster RE. Diffusion of gases across the alveolar membrane, In: Fishman AP, ed. Handbook of Physiology. Bethesda, American Physiological Society, 1987; pp. 71-88. 6 Borland CD, Dunningham H, Bottrill F, et al. Significant blood resistance to nitric oxide transfer in the lung. J Appl Physiol 2011; 108: 1052-1060. 7 Carlsen E, Comroe JH Jr. The rate of uptake of carbon monoxide and of nitric oxide by normal human erythrocytes and experimentally produced spherocytes. J Gen Physiol 1958; 42: 83-107. 8 Martinot JB, Mulè M, de Bisschop C, et al. Lung membrane conductance and capillary volume derived from the NO and CO transfer in high-altitude newcomers. J Appl Physiol (1985) 2013; 115: 157-166. 9 Aguilaniu B, Maitre J, Glénet S, et al. European reference equations for CO and NO lung transfer. Eur Respir J 2008; 31: 1091-1097. 10 Weibel ER, Federspiel WJ, Fryder-Doffey F, et al. Morphometric model for pulmonary diffusing capacity. I. Membrane diffusing capacity.
A63. THE UNSTOPPABLE PROGRESS IN LYMPHANGIOLEIOMYOMATOSIS RESEARCH, 2010
University of Sao Paulo Medical School, Sao Paulo, Brazil, University of Sao Paulo Medical School... more University of Sao Paulo Medical School, Sao Paulo, Brazil, University of Sao Paulo Medical School, sao paulo, Brazil 1 ... Corresponding author's email: [email protected] ... Lymphangioleiomyomatosis (LAM) is a rare disease, usually evolving to chronic respiratory failure, characterized by ...
The functional evaluation has become increasingly important in the understanding and management o... more The functional evaluation has become increasingly important in the understanding and management of patients with interstitial lung diseases. The cardiopulmonary exercise test and the six-minute walk test (6MWT), through their isolated variables, have been used to do this evaluation, with some limitations.
Nas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-s... more Nas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-se receptores de estrogênio e progesterona, de modo que o tratamento anti-hormonal pode ser considerado uma opção, mas ainda com resultados controversos. O objetivo deste trabalho foi avaliar retrospectivamente parâmetros hormonais e espirométricos em nove mulheres com LAM após o tratamento com goserelina por um ano. Houve um aumento médio de 80 mL e 130 mL, respectivamente, em VEF 1 e CVF, assim como bloqueio hormonal efetivo. Ainda não se pode excluir um potencial efeito favorável da utilização de análogos de hormônio liberador de gonadotrofina em pacientes com LAM, reforçando a necessidade de ensaios randomizados.
Introduction: Positive responses to bronchodilators (BDs) on spirometry can be found in up to 30%... more Introduction: Positive responses to bronchodilators (BDs) on spirometry can be found in up to 30% of patients with lymphangioleiomyomatosis (LAM). However, no previous studies have investigated the impact of BDs on exercise outcomes, including dynamic hyperinflation (DH). Methods: A randomised, double-blind, placebo-controlled, crossover trial was conducted on 38 patients with LAM, comparing inhaled placebo versus salbutamol. Pulmonary function tests and a cycle endurance test at 75% of the maximal work capacity, with evaluation of DH by serial measurement of inspiratory capacity (IC), which was the primary endpoint, were performed after each intervention. Results: Although salbutamol produced a slight improvement in airway obstruction, compared with placebo, there was no significant variation in resting IC or air trapping. A total of 18% of the patients met the criteria for a positive response to BD. During submaximal exercise, BD did not reduce DH or dyspnoea nor did it improve exercise tolerance in the entire population. In addition, BD produced only slight improvement in FEV 1 and air trapping in patients who had DH during incremental cardiopulmonary exercise testing, without the beneficial effects on exercise outcomes. Conclusions: Although salbutamol produced a slight improvement in airway obstruction, it did not lead to a reduction in DH or increase in exercise tolerance in patients with LAM. Clinical trial registration: Brazilian Registry of Clinical Trials (ReBEC); www.ensaiosclinicos. gov.br; registration number: RBR-49sk2j. ª
American Journal of Respiratory and Critical Care Medicine, 2012
Rationale: Lymphangioleiomyomatosis (LAM) is characterized by exercise performance impairment. Al... more Rationale: Lymphangioleiomyomatosis (LAM) is characterized by exercise performance impairment. Although airflow limitation is common, no previous studies have evaluated the prevalence and impact of dynamic hyperinflation (DH) in LAM. Objectives: To investigate the dynamic responses during maximal exercise and the prevalence, predictors, and repercussions of DH in LAM. Methods: Forty-two patients with LAM performed symptom-limited incremental cycle exercise and pulmonary functions tests (PFTs) and were compared with 10 age-matched healthy women. Dyspnea intensity, inspiratory capacity, oxygen saturation, and cardiac, metabolic, and respiratory variables were assessed during exercise. Patients with LAM also performed a 6-minute walk test (6MWT). Measurements and Main Results: Patients with LAM had higher baseline dyspnea, poorer quality of life, obstructive pattern, air trapping, and reduced diffusing capacity of carbon monoxide in PFTs. Although they had the same level of regular physical activity, their maximal exercise performance was reduced and was associated with ventilatory limitation, greater desaturation, and dyspnea. The prevalence of DH was high in LAM (55%), even in patients with mild spirometric abnormalities, and was correlated with airflow obstruction, air trapping, and diffusing capacity of carbon monoxide. Compared with the non-DH subgroup, the patients who developed DH had a ventilatory limitation contributing to exercise cessation on cycling and higher desaturation and dyspnea intensity during the 6MWT. Conclusions: Ventilatory limitation and gas exchange impairment are important causes of exercise limitation in LAM. DH is frequent in LAM, even in patients with mild spirometric abnormalities. DH was associated with the severity of disease, higher dyspnea, and lower oxygen saturation. In the 6MWT, desaturation and dyspnea were greater in patients with DH.
Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung paren... more On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.
... PM / Area B, Hall G (First Level), Morial Convention Center Bronchiolitis Obliterans With Org... more ... PM / Area B, Hall G (First Level), Morial Convention Center Bronchiolitis Obliterans With Organizing Pneumonia (BOOP) As Pulmonary Manifestation Of Multiple Myeloma SZ Rached , RA Athanazio , , T. Mauad , RA Kairalla , CR Carvalho 1 2 BG Baldi3 4 3 3 ...
Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of condit... more 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.
Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue dise... more Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue disease (MCTD). However, little is known about the long-term progression of ILD in MCTD. The aims of this study were to describe pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results in long-term MCTD patients, to measure changes in PFT and HRCT results over a 10-year period, and to ascertain correlations in functional and imaging data. In this retrospective cohort study, comparison between baseline and follow-up PFT and HRCT data was performed for 39 unselected consecutive MCTD patients. At baseline, 51% of the patients had abnormal PFTs. Forced vital capacity (FVC) was slightly reduced at baseline (77% of predicted), but remained stable after 10 years. A relative decrease of 15% in the diffusion capacity for carbon monoxide (DLCO) was detected (from 84% to 71% of predicted, p<0.001). The median lower lobes ILD-HRCT score progressed from 7.5% at baseline to...
To describe the characteristics of a cohort of patients with lung-dominant connective tissue dise... more To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt; 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.
1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung... more 1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung transfer ratio: physiological implications. J Physiol 2007; 582: 767-775. 2 Hughes JM, van der Lee I. The TL,NO/TL,CO ratio in pulmonary function test interpretation. Eur Respir J 2013; 41: 453-461. 3 Guenard H, Varene N, Vaida P. Determination of lung capillary blood volume and membrane diffusing capacity in man by the measurements of NO and CO transfer. Respir Physiol 1987; 70: 113-120. 4 Roughton FJ, Forster RE. Relative importance of diffusion and chemical reaction rates in determining rate of exchange of gases in the human lung, with special reference to true diffusing capacity of pulmonary membrane and volume of blood in the lung capillaries. J Appl Physiol 1957; 11: 290-302. 5 Forster RE. Diffusion of gases across the alveolar membrane, In: Fishman AP, ed. Handbook of Physiology. Bethesda, American Physiological Society, 1987; pp. 71-88. 6 Borland CD, Dunningham H, Bottrill F, et al. Significant blood resistance to nitric oxide transfer in the lung. J Appl Physiol 2011; 108: 1052-1060. 7 Carlsen E, Comroe JH Jr. The rate of uptake of carbon monoxide and of nitric oxide by normal human erythrocytes and experimentally produced spherocytes. J Gen Physiol 1958; 42: 83-107. 8 Martinot JB, Mulè M, de Bisschop C, et al. Lung membrane conductance and capillary volume derived from the NO and CO transfer in high-altitude newcomers. J Appl Physiol (1985) 2013; 115: 157-166. 9 Aguilaniu B, Maitre J, Glénet S, et al. European reference equations for CO and NO lung transfer. Eur Respir J 2008; 31: 1091-1097. 10 Weibel ER, Federspiel WJ, Fryder-Doffey F, et al. Morphometric model for pulmonary diffusing capacity. I. Membrane diffusing capacity.
Page 1. / Thematic Poster Session / Wednesday, May 19/8:15 AM-4:00 PM / Area D36 SARCOIDOSIS: TRA... more Page 1. / Thematic Poster Session / Wednesday, May 19/8:15 AM-4:00 PM / Area D36 SARCOIDOSIS: TRANSLATIONAL APPROACHES E, Hall G (First Level), Morial Convention Center Is Silica Exposure A Precipitating Agent Of Pulmonary Sarcoidosis? ...
INTRODUCTION The evaluation of Idiopathic Interstitial Pneumonia (IIP) patients requires active s... more INTRODUCTION The evaluation of Idiopathic Interstitial Pneumonia (IIP) patients requires active sought of clinical and laboratorial features of Connective Tissue Disease (CTD). Autoantibodies positivity is routinely evaluated in this population, but there is no agreement on when and at which frequency those tests should be repeated if negative or inconclusive at initial evaluation. We report an IIP patient in whom we performed repeated serological measures, suggesting that ILD might precede CTD features in several years. CASE A 30-year male was evaluated at our institution in February 1994 for cough and mild dyspnea initiated a year before. Examination showed bibasilar crackles and oxymetry 97% at room air. Pulmonary function tests showed moderate restrictive pattern (FVC 51%pred) and reduced DLCO (45%pred). Chest CT showed peripheral reticulate, ground-glass opacities (GGO) and dilated, fluid-filled esophagus. Esophageal manometry confirmed dismotility. Antinuclear antibody (ANA) w...
INTRODUCTION Patients with ILD and autoantibody (AutoAb) positivity probably have different chara... more INTRODUCTION Patients with ILD and autoantibody (AutoAb) positivity probably have different characteristics from Idiopathic Interstitial Pneumonias (IIP) regarding prognosis and natural history. Lung-Dominant Connective Tissue Disease (LD-CTD) is characterized by interstitial lung disease (ILD) in association with clinical features suggesting CTD, without meeting accepted rheumatologic criteria. However, previous studies applied heterogeneous criteria of LD-CTD concerning the role of CTD extrathoracic features in those patients. We hypothesized that ILD patients with isolated AutoAb positivity might be different from those with associated extrathoracic features. Therefore, we designed a retrospective cohort study to compare LD-CTD patients with and without extrathoracic features. METHODS We selected from our database ILD patients with positive ANA≥1/160 and/or specific AutoAb. We excluded definitive CTD and ILD's with known most probable etiology. The presence of extrathoracic f...
que a posição obtida e o valor do indicador, a volta do JBP ao relatório anual da base de dados g... more que a posição obtida e o valor do indicador, a volta do JBP ao relatório anual da base de dados geradora do Fator de Impacto demonstra que o caminho trilhado ao longo dos últimos anos sempre foi pautado pela divulgação da ciência de qualidade na área respiratória. Conforme mencionado, o Fator de Impacto do JBP, pelo mecanismo através do qual é feito seu cálculo, ainda espelhará o efeito da penalidade sofrida ao longo de pelo menos os próximos dois anos; ainda assim, o retorno do JBP consolida sua posição dentre as revistas de relevância para a divulgação da área de medicina respiratória no mundo.
1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung... more 1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung transfer ratio: physiological implications. J Physiol 2007; 582: 767-775. 2 Hughes JM, van der Lee I. The TL,NO/TL,CO ratio in pulmonary function test interpretation. Eur Respir J 2013; 41: 453-461. 3 Guenard H, Varene N, Vaida P. Determination of lung capillary blood volume and membrane diffusing capacity in man by the measurements of NO and CO transfer. Respir Physiol 1987; 70: 113-120. 4 Roughton FJ, Forster RE. Relative importance of diffusion and chemical reaction rates in determining rate of exchange of gases in the human lung, with special reference to true diffusing capacity of pulmonary membrane and volume of blood in the lung capillaries. J Appl Physiol 1957; 11: 290-302. 5 Forster RE. Diffusion of gases across the alveolar membrane, In: Fishman AP, ed. Handbook of Physiology. Bethesda, American Physiological Society, 1987; pp. 71-88. 6 Borland CD, Dunningham H, Bottrill F, et al. Significant blood resistance to nitric oxide transfer in the lung. J Appl Physiol 2011; 108: 1052-1060. 7 Carlsen E, Comroe JH Jr. The rate of uptake of carbon monoxide and of nitric oxide by normal human erythrocytes and experimentally produced spherocytes. J Gen Physiol 1958; 42: 83-107. 8 Martinot JB, Mulè M, de Bisschop C, et al. Lung membrane conductance and capillary volume derived from the NO and CO transfer in high-altitude newcomers. J Appl Physiol (1985) 2013; 115: 157-166. 9 Aguilaniu B, Maitre J, Glénet S, et al. European reference equations for CO and NO lung transfer. Eur Respir J 2008; 31: 1091-1097. 10 Weibel ER, Federspiel WJ, Fryder-Doffey F, et al. Morphometric model for pulmonary diffusing capacity. I. Membrane diffusing capacity.
A63. THE UNSTOPPABLE PROGRESS IN LYMPHANGIOLEIOMYOMATOSIS RESEARCH, 2010
University of Sao Paulo Medical School, Sao Paulo, Brazil, University of Sao Paulo Medical School... more University of Sao Paulo Medical School, Sao Paulo, Brazil, University of Sao Paulo Medical School, sao paulo, Brazil 1 ... Corresponding author&amp;#x27;s email: [email protected] ... Lymphangioleiomyomatosis (LAM) is a rare disease, usually evolving to chronic respiratory failure, characterized by ...
The functional evaluation has become increasingly important in the understanding and management o... more The functional evaluation has become increasingly important in the understanding and management of patients with interstitial lung diseases. The cardiopulmonary exercise test and the six-minute walk test (6MWT), through their isolated variables, have been used to do this evaluation, with some limitations.
Nas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-s... more Nas células musculares lisas atípicas características da linfangioleiomiomatose (LAM) encontram-se receptores de estrogênio e progesterona, de modo que o tratamento anti-hormonal pode ser considerado uma opção, mas ainda com resultados controversos. O objetivo deste trabalho foi avaliar retrospectivamente parâmetros hormonais e espirométricos em nove mulheres com LAM após o tratamento com goserelina por um ano. Houve um aumento médio de 80 mL e 130 mL, respectivamente, em VEF 1 e CVF, assim como bloqueio hormonal efetivo. Ainda não se pode excluir um potencial efeito favorável da utilização de análogos de hormônio liberador de gonadotrofina em pacientes com LAM, reforçando a necessidade de ensaios randomizados.
Introduction: Positive responses to bronchodilators (BDs) on spirometry can be found in up to 30%... more Introduction: Positive responses to bronchodilators (BDs) on spirometry can be found in up to 30% of patients with lymphangioleiomyomatosis (LAM). However, no previous studies have investigated the impact of BDs on exercise outcomes, including dynamic hyperinflation (DH). Methods: A randomised, double-blind, placebo-controlled, crossover trial was conducted on 38 patients with LAM, comparing inhaled placebo versus salbutamol. Pulmonary function tests and a cycle endurance test at 75% of the maximal work capacity, with evaluation of DH by serial measurement of inspiratory capacity (IC), which was the primary endpoint, were performed after each intervention. Results: Although salbutamol produced a slight improvement in airway obstruction, compared with placebo, there was no significant variation in resting IC or air trapping. A total of 18% of the patients met the criteria for a positive response to BD. During submaximal exercise, BD did not reduce DH or dyspnoea nor did it improve exercise tolerance in the entire population. In addition, BD produced only slight improvement in FEV 1 and air trapping in patients who had DH during incremental cardiopulmonary exercise testing, without the beneficial effects on exercise outcomes. Conclusions: Although salbutamol produced a slight improvement in airway obstruction, it did not lead to a reduction in DH or increase in exercise tolerance in patients with LAM. Clinical trial registration: Brazilian Registry of Clinical Trials (ReBEC); www.ensaiosclinicos. gov.br; registration number: RBR-49sk2j. ª
American Journal of Respiratory and Critical Care Medicine, 2012
Rationale: Lymphangioleiomyomatosis (LAM) is characterized by exercise performance impairment. Al... more Rationale: Lymphangioleiomyomatosis (LAM) is characterized by exercise performance impairment. Although airflow limitation is common, no previous studies have evaluated the prevalence and impact of dynamic hyperinflation (DH) in LAM. Objectives: To investigate the dynamic responses during maximal exercise and the prevalence, predictors, and repercussions of DH in LAM. Methods: Forty-two patients with LAM performed symptom-limited incremental cycle exercise and pulmonary functions tests (PFTs) and were compared with 10 age-matched healthy women. Dyspnea intensity, inspiratory capacity, oxygen saturation, and cardiac, metabolic, and respiratory variables were assessed during exercise. Patients with LAM also performed a 6-minute walk test (6MWT). Measurements and Main Results: Patients with LAM had higher baseline dyspnea, poorer quality of life, obstructive pattern, air trapping, and reduced diffusing capacity of carbon monoxide in PFTs. Although they had the same level of regular physical activity, their maximal exercise performance was reduced and was associated with ventilatory limitation, greater desaturation, and dyspnea. The prevalence of DH was high in LAM (55%), even in patients with mild spirometric abnormalities, and was correlated with airflow obstruction, air trapping, and diffusing capacity of carbon monoxide. Compared with the non-DH subgroup, the patients who developed DH had a ventilatory limitation contributing to exercise cessation on cycling and higher desaturation and dyspnea intensity during the 6MWT. Conclusions: Ventilatory limitation and gas exchange impairment are important causes of exercise limitation in LAM. DH is frequent in LAM, even in patients with mild spirometric abnormalities. DH was associated with the severity of disease, higher dyspnea, and lower oxygen saturation. In the 6MWT, desaturation and dyspnea were greater in patients with DH.
Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung paren... more On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.
... PM / Area B, Hall G (First Level), Morial Convention Center Bronchiolitis Obliterans With Org... more ... PM / Area B, Hall G (First Level), Morial Convention Center Bronchiolitis Obliterans With Organizing Pneumonia (BOOP) As Pulmonary Manifestation Of Multiple Myeloma SZ Rached , RA Athanazio , , T. Mauad , RA Kairalla , CR Carvalho 1 2 BG Baldi3 4 3 3 ...
Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of condit... more 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.
Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue dise... more Interstitial lung disease (ILD) is highly prevalent in patients with mixed connective tissue disease (MCTD). However, little is known about the long-term progression of ILD in MCTD. The aims of this study were to describe pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results in long-term MCTD patients, to measure changes in PFT and HRCT results over a 10-year period, and to ascertain correlations in functional and imaging data. In this retrospective cohort study, comparison between baseline and follow-up PFT and HRCT data was performed for 39 unselected consecutive MCTD patients. At baseline, 51% of the patients had abnormal PFTs. Forced vital capacity (FVC) was slightly reduced at baseline (77% of predicted), but remained stable after 10 years. A relative decrease of 15% in the diffusion capacity for carbon monoxide (DLCO) was detected (from 84% to 71% of predicted, p<0.001). The median lower lobes ILD-HRCT score progressed from 7.5% at baseline to...
To describe the characteristics of a cohort of patients with lung-dominant connective tissue dise... more To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). This was a retrospective study of patients with interstitial lung disease (ILD), positive antinuclear antibody (ANA) results (≥ 1/320), with or without specific autoantibodies, and at least one clinical feature suggestive of connective tissue disease (CTD). Of the 1,998 patients screened, 52 initially met the criteria for a diagnosis of LD-CTD: 37% were male; the mean age at diagnosis was 56 years; and the median follow-up period was 48 months. During follow-up, 8 patients met the criteria for a definitive diagnosis of a CTD. The remaining 44 patients comprised the LD-CTD group, in which the most prevalent extrathoracic features were arthralgia, gastroesophageal reflux disease, and Raynaud&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s phenomenon. The most prevalent autoantibodies in this group were ANA (89%) and anti-SSA (anti-Ro, 27%). The mean baseline and final FVC was 69.5% and 74.0% of the predicted values, respectively (p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt; 0.05). Nonspecific interstitial pneumonia and usual interstitial pneumonia patterns were found in 45% and 9% of HRCT scans, respectively; 36% of the scans were unclassifiable. A similar prevalence was noted in histological samples. Diffuse esophageal dilatation was identified in 52% of HRCT scans. Nailfold capillaroscopy was performed in 22 patients; 17 showed a scleroderma pattern. In our LD-CTD group, there was predominance of females and the patients showed mild spirometric abnormalities at diagnosis, with differing underlying ILD patterns that were mostly unclassifiable on HRCT and by histology. We found functional stability on follow-up. Esophageal dilatation on HRCT and scleroderma pattern on nailfold capillaroscopy were frequent findings and might come to serve as diagnostic criteria.
1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung... more 1 Glénet SN, De Bisschop C, Vargas F, et al. Deciphering the nitric oxide to carbon monoxide lung transfer ratio: physiological implications. J Physiol 2007; 582: 767-775. 2 Hughes JM, van der Lee I. The TL,NO/TL,CO ratio in pulmonary function test interpretation. Eur Respir J 2013; 41: 453-461. 3 Guenard H, Varene N, Vaida P. Determination of lung capillary blood volume and membrane diffusing capacity in man by the measurements of NO and CO transfer. Respir Physiol 1987; 70: 113-120. 4 Roughton FJ, Forster RE. Relative importance of diffusion and chemical reaction rates in determining rate of exchange of gases in the human lung, with special reference to true diffusing capacity of pulmonary membrane and volume of blood in the lung capillaries. J Appl Physiol 1957; 11: 290-302. 5 Forster RE. Diffusion of gases across the alveolar membrane, In: Fishman AP, ed. Handbook of Physiology. Bethesda, American Physiological Society, 1987; pp. 71-88. 6 Borland CD, Dunningham H, Bottrill F, et al. Significant blood resistance to nitric oxide transfer in the lung. J Appl Physiol 2011; 108: 1052-1060. 7 Carlsen E, Comroe JH Jr. The rate of uptake of carbon monoxide and of nitric oxide by normal human erythrocytes and experimentally produced spherocytes. J Gen Physiol 1958; 42: 83-107. 8 Martinot JB, Mulè M, de Bisschop C, et al. Lung membrane conductance and capillary volume derived from the NO and CO transfer in high-altitude newcomers. J Appl Physiol (1985) 2013; 115: 157-166. 9 Aguilaniu B, Maitre J, Glénet S, et al. European reference equations for CO and NO lung transfer. Eur Respir J 2008; 31: 1091-1097. 10 Weibel ER, Federspiel WJ, Fryder-Doffey F, et al. Morphometric model for pulmonary diffusing capacity. I. Membrane diffusing capacity.
Page 1. / Thematic Poster Session / Wednesday, May 19/8:15 AM-4:00 PM / Area D36 SARCOIDOSIS: TRA... more Page 1. / Thematic Poster Session / Wednesday, May 19/8:15 AM-4:00 PM / Area D36 SARCOIDOSIS: TRANSLATIONAL APPROACHES E, Hall G (First Level), Morial Convention Center Is Silica Exposure A Precipitating Agent Of Pulmonary Sarcoidosis? ...
INTRODUCTION The evaluation of Idiopathic Interstitial Pneumonia (IIP) patients requires active s... more INTRODUCTION The evaluation of Idiopathic Interstitial Pneumonia (IIP) patients requires active sought of clinical and laboratorial features of Connective Tissue Disease (CTD). Autoantibodies positivity is routinely evaluated in this population, but there is no agreement on when and at which frequency those tests should be repeated if negative or inconclusive at initial evaluation. We report an IIP patient in whom we performed repeated serological measures, suggesting that ILD might precede CTD features in several years. CASE A 30-year male was evaluated at our institution in February 1994 for cough and mild dyspnea initiated a year before. Examination showed bibasilar crackles and oxymetry 97% at room air. Pulmonary function tests showed moderate restrictive pattern (FVC 51%pred) and reduced DLCO (45%pred). Chest CT showed peripheral reticulate, ground-glass opacities (GGO) and dilated, fluid-filled esophagus. Esophageal manometry confirmed dismotility. Antinuclear antibody (ANA) w...
INTRODUCTION Patients with ILD and autoantibody (AutoAb) positivity probably have different chara... more INTRODUCTION Patients with ILD and autoantibody (AutoAb) positivity probably have different characteristics from Idiopathic Interstitial Pneumonias (IIP) regarding prognosis and natural history. Lung-Dominant Connective Tissue Disease (LD-CTD) is characterized by interstitial lung disease (ILD) in association with clinical features suggesting CTD, without meeting accepted rheumatologic criteria. However, previous studies applied heterogeneous criteria of LD-CTD concerning the role of CTD extrathoracic features in those patients. We hypothesized that ILD patients with isolated AutoAb positivity might be different from those with associated extrathoracic features. Therefore, we designed a retrospective cohort study to compare LD-CTD patients with and without extrathoracic features. METHODS We selected from our database ILD patients with positive ANA≥1/160 and/or specific AutoAb. We excluded definitive CTD and ILD's with known most probable etiology. The presence of extrathoracic f...
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