Brazilian Journal of Pulmonology

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

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Control measures to trace ≤ 15-year-old contacts of index cases of active pulmonary tuberculosis

Atualização no diagnóstico e tratamento da fibrose pulmonar idiopática

José Baddini-Martinez1, Bruno Guedes Baldi2, Cláudia Henrique da Costa3, Sérgio Jezler4, Mariana Silva Lima5, Rogério Rufino3,6

J Bras Pneumol.2015;41(5):454-466

Abstract PDF PT PDF EN Portuguese Text

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

 


Keywords: Idiopathic pulmonary fibrosis/diagnosis; Idiopathic pulmonary fibrosis/therapy; Idiopathic pulmonary fibrosis/rehabilitation.

 


Evaluation of articles on pulmonology published in Brazilian journals other than the Brazilian Journal of Pulmonology

Avaliação dos artigos de pneumologia publicados em periódicos brasileiros além do Jornal Brasileiro de Pneumologia

Bruno Guedes Baldi, Carlos Roberto Ribeiro

J Bras Pneumol.2011;37(6):801-808

Abstract PDF PT PDF EN Portuguese Text

In Brazil, research on pulmonology has become increasingly more visible in recent years. In addition to the Brazilian Journal of Pulmonology, other journals have contributed to that by publishing relevant articles in this area. The objective of this article was to briefly report the most relevant studies on pulmonology that were published in other important Brazilian journals between 2009 and 2010. Altogether, there were 56 articles related to the various subareas that compose the field of respiratory diseases.

 


Keywords: Pulmonary medicine; Research; Brazil.

 


Lung cysts in chronic paracoccidioidomycosis

Cistos pulmonares na paracoccidioidomicose crônica

André Nathan Costa, Edson Marchiori, Gil Benard, Mariana Sponholz Araújo, Bruno Guedes Baldi, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2013;39(3):368-372

Abstract PDF PT PDF EN Portuguese Text

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.

 


Keywords: Paracoccidioidomycosis; Cysts; Multidetector computed tomography.

 


Lung-dominant connective tissue disease among patients with inter-stitial lung disease: prevalence, functional stability, and common extrathoracic features

Colagenose pulmão dominante em pacientes com doença pulmonar intersticial: prevalência, estabilidade funcional e manifestações extratorácicas comuns

Daniel Antunes Silva Pereira, Olívia Meira Dias, Guilherme Eler de Almeida, Mariana Sponholz Araujo, Letícia Barbosa Kawano-Dourado, Bruno Guedes Baldi, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2015;41(2):151-160

Abstract PDF PT PDF EN Portuguese Text

Objective: To describe the characteristics of a cohort of patients with lung-dominant connective tissue disease (LD-CTD). Methods: 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). Results: 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'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 > 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. Conclusions: 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.

 


Keywords: Idiopathic interstitial pneumonias; Autoantibodies; Connective tissue diseases; Autoimmunity.

 


Combined pulmonary fibrosis and emphysema: an increasingly recognized condition

Combinação de fibrose pulmonar e enfisema: uma doença cada vez mais reconhecida

Olívia Meira Dias, Bruno Guedes Baldi, André Nathan Costa, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2014;40(3):304-312

Abstract PDF PT PDF EN Portuguese Text

Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized in the literature. Patients with CPFE are usually heavy smokers or former smokers with concomitant lower lobe fibrosis and upper lobe emphysema on chest HRCT scans. They commonly present with severe breathlessness and low DLCO, despite spirometry showing relatively preserved lung volumes. Moderate to severe pulmonary arterial hypertension is common in such patients, who are also at an increased risk of developing lung cancer. Unfortunately, there is currently no effective treatment for CPFE. In this review, we discuss the current knowledge of the pathogenesis, clinical characteristics, and prognostic factors of CPFE. Given that most of the published data on CPFE are based on retrospective analysis, more studies are needed in order to address the role of emphysema and its subtypes; the progression of fibrosis/emphysema and its correlation with inflammation; treatment options; and prognosis.

 


Keywords: Pulmonary fibrosis; Emphysema; Hypertension, pulmonary; Lung diseases, interstitial.

 


How and why to review articles for the Jornal Brasileiro de Pneumologia

Como e por que revisar artigos para o Jornal Brasileiro de Pneumologia

Bruno Guedes Baldi1,2,a, Pedro Rodrigues Genta1,3,b

J Bras Pneumol.2019;45(5):e20190319-e20190319

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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.

 


Publication of the impact factor of the Brazilian Journal of Pulmonology: a milestone on a long and arduous journey

Divulgação do fator de impacto do Jornal Brasileiro de Pneumologia: consolidação de um longo e árduo trabalho

Carlos Roberto Ribeiro Carvalho, Bruno Guedes Baldi, Carlos Viana Poyares Jardim, Pedro Caruso

J Bras Pneumol.2012;38(4):417-418

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Adalimumab-induced acute interstitial lung disease in a patient with rheumatoid arthritis

Doença pulmonar intersticial aguda induzida por adalimumabe em paciente com artrite reumatoide

Olívia Meira Dias, Daniel Antunes Silva Pereira, Bruno Guedes Baldi, André Nathan Costa, Rodrigo Abensur Athanazio, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2014;40(1):77-81

Abstract PDF PT PDF EN Portuguese Text

The use of immunobiological agents for the treatment of autoimmune diseases is increasing in medical practice. Anti-TNF therapies have been increasingly used in refractory autoimmune diseases, especially rheumatoid arthritis, with promising results. However, the use of such therapies has been associated with an increased risk of developing other autoimmune diseases. In addition, the use of anti-TNF agents can cause pulmonary complications, such as reactivation of mycobacterial and fungal infections, as well as sarcoidosis and other interstitial lung diseases (ILDs). There is evidence of an association between ILD and the use of anti-TNF agents, etanercept and infliximab in particular. Adalimumab is the newest drug in this class, and some authors have suggested that its use might induce or exacerbate preexisting ILDs. In this study, we report the first case of acute ILD secondary to the use of adalimumab in Brazil, in a patient with rheumatoid arthritis and without a history of ILD.

 


Keywords: Lung diseases, interstitial; Arthritis, rheumatoid; Antirheumatic agents; Antibodies, monoclonal, humanized/adverse effects.

 


Diffuse cystic lung diseases: differential diagnosis

Doenças pulmonares císticas difusas: diagnóstico diferencial

Bruno Guedes Baldi1, Carlos Roberto Ribeiro Carvalho1, Olívia Meira Dias1, Edson Marchiori2,3, Bruno Hochhegger4,5,6

J Bras Pneumol.2017;43(2):140-149

Abstract PDF PT PDF EN Portuguese Text

Diffuse cystic lung diseases are characterized by cysts in more than one lung lobe, the cysts originating from various mechanisms, including the expansion of the distal airspaces due to airway obstruction, necrosis of the airway walls, and parenchymal destruction. The progression of these diseases is variable. One essential tool in the evaluation of these diseases is HRCT, because it improves the characterization of pulmonary cysts (including their distribution, size, and length) and the evaluation of the regularity of the cyst wall, as well as the identification of associated pulmonary and extrapulmonary lesions. When combined with clinical and laboratory findings, HRCT is often sufficient for the etiological definition of diffuse lung cysts, avoiding the need for lung biopsy. The differential diagnoses of diffuse cystic lung diseases are myriad, including neoplastic, inflammatory, and infectious etiologies. Pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, lymphocytic interstitial pneumonia, and follicular bronchiolitis are the most common diseases that produce this CT pattern. However, new diseases have been included as potential determinants of this pattern.

 


Keywords: Cysts; Diagnosis, differential; Lung diseases, interstitial; Tomography, X-ray computed.

 


Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade

Doxiciclina em pacientes com linfangioleiomiomatose: segurança e eficácia no bloqueio de metaloproteinases

Suzana Pinheiro Pimenta, Bruno Guedes Baldi, Milena Marques Pagliarelli Acencio, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2011;37(4):424-430

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Objective: To determine the frequency of near-fatal asthma in a group of severe asthma patients, as well as the clinical characteristics and prognosis of these patients within a one-year follow-up period. Methods: A prospective study involving 731 low-income patients with severe asthma treated at a referral outpatient clinic located in the city of Salvador, Brazil. The patients were submitted to spirometry at admission, received medications for asthma, and were monitored regarding the frequency of asthma exacerbations during the follow-up period. A subsample of 511 patients also completed questionnaires regarding asthma symptoms and asthma-related quality of life. Results: Of the 731 patients studied, 563 (77%) were female. The median age was 47 years, and 12% were illiterate. Most of the patients had rhinitis, and 70 patients (10%) reported near-fatal asthma prior to admission. Of these 70 patients, 41 (59%) reported having been intubated previously. The patients reporting a history of near-fatal asthma at admission were more likely to have asthma exacerbations during the follow-up period and to respond poorly to therapy than were those not reporting such a history. At the end of the follow-up period, the scores on the two questionnaires were similar between the two groups of patients. Conclusions: The frequency of near-fatal asthma was high in this group of low-income patients with severe asthma. The patients with a history of near-fatal asthma had a worse prognosis than did those without such a history, although both groups had received the same kind of treatment. Curiously, the intensity of symptoms and the quality of life at the end of the study were similar between the two groups.

 


Keywords: Asthma/prevention and control; Asthma/complications; Quality of life; Prognosis.

 


Doxycycline use in patients with lymphangioleiomyomatosis: biomarkers and pulmonary function response

Doxiciclina em pacientes com linfangioleiomiomatose: biomarcadores e resposta funcional pulmonar

Suzana Pinheiro Pimenta, Bruno Guedes Baldi, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2013;39(1):5-15

Abstract PDF PT PDF EN Appendix

Objective: To assess blockade of matrix metalloproteinase (MMP)-2 and MMP-9, as well as the variation in FEV1, in patients with lymphangioleiomyomatosis (LAM) treated with doxycycline (a known MMP inhibitor) for 12 months. Methods: An open-label, single-arm, interventional clinical trial in which LAM patients received doxycycline (100 mg/day) for 12 months. Patients underwent full pulmonary function testing, a six-minute walk test, and quality of life assessment, as well as blood and urine sampling for quantification of MMP-2, MMP-9, and VEGF-D levels-at baseline, as well as at 6 and 12 months after the initiation of doxycycline. Results: Thirty-one LAM patients received doxycycline for 12 months. Although there was effective blockade of urinary MMP-9 and serum MMP-2 after treatment, there were no significant differences between pre‑and post-doxycycline serum levels of MMP-9 and VEGF-D. On the basis of their response to doxycycline (as determined by the variation in FEV1), the patients were divided into two groups: the doxycycline-responder (doxy-R) group (n = 13); and the doxycycline-nonresponder (doxy-NR) group (n = 18). The patients with mild spirometric abnormalities responded better to doxycycline. The most common side effects were mild epigastric pain, nausea, and diarrhea. Conclusions: In patients with LAM, doxycycline treatment results in effective MMP blockade, as well as in improved lung function and quality of life in those with less severe disease. However, these benefits do not seem to be related to the MMP blockade, raising the hypothesis that there is a different mechanism of action. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-6g8yz9 [http://www.ensaiosclinicos.gov.br])

 


Keywords: Lymphangioleiomyomatosis; Doxycycline; Matrix metalloproteinases; Respiratory function tests.

 


Evolution of pulmonary function after treatment with goserelin in patients with lymphangioleiomyomatosis

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

Bruno Guedes Baldi, Pedro Medeiros Junior, Suzana Pinheiro Pimenta, Roberto Iglesias Lopes, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2011;37(3):375-379

Abstract PDF PT PDF EN Portuguese Text

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.

 


Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin.

 


Idiopathic pulmonary fibrosis can be a transient diagnosis

Fibrose pulmonar idiopática pode ser um diagnóstico transitório

Martina Rodrigues de Oliveira1, Daniel Antunes Silva Pereira1, Olívia Meira Dias1, Ronaldo Adib Kairalla1, Carlos Roberto Ribeiro Carvalho1, Bruno Guedes Baldi1

J Bras Pneumol.2016;42(1):74-75

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Dynamic hyperinflation on exertion: much remains unknown

Hiperinsuflação dinâmica no esforço: ainda muito a ser esclarecido

André Luís Pereira de Albuquerque, Bruno Guedes Baldi

J Bras Pneumol.2012;38(1):1-3

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Interpretation of autoantibody positivity in interstitial lung disease and lung-dominant connective tissue disease

Interpretação da positividade de autoanticorpos na doença pulmonar intersticial e colagenose pulmão dominante

Daniel Antunes Silva Pereira, Alexandre de Melo Kawassaki, Bruno Guedes Baldi

J Bras Pneumol.2013;39(6):728-741

Abstract PDF PT PDF EN Portuguese Text

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild or benign symptoms of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

 


Keywords: Idiopathic interstitial pneumonias; Autoantibodies; Connective tissue diseases; Autoimmune diseases; Diagnosis, differential.

 


Jornal Brasileiro de Pneumologia and Sociedade Brasileira de Pneumologia e Tisiologia: perspectives for the next four years

Jornal Brasileiro de Pneumologia e Sociedade Brasileira de Pneumologia e Tisiologia: perspectivas para os próximos quatro anos

Bruno Guedes Baldi1,2,a, José Miguel Chatkin3,4,b

J Bras Pneumol.2019;45(1):e20190028-e20190028

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Pulmonary reperfusion injury by aortic occlusion: experimental model in rats

Lesão pulmonar de reperfusão por oclusão da aorta abdominal: modelo experimental em ratos

Bruno do Valle Pinheiro, Cândida Maria Moreira Horta, Bruno Guedes Baldi, Leonardo de Lucca Schiavon, Ângela Maria Gollner, Júlio César Abreu de Oliveira

J Bras Pneumol.2000;26(4):163-168

Abstract PDF PT

Introduction: Surgical procedures requiring aortic occlusion have been associated with pulmonary reperfusion injury. The authors studied the pulmonary edema in a reperfusion injury model by aortic occlusion in rats. Material and methods: Thirty-three Wistar rats were anesthetized with intraperitoneal injection of sodium pentobarbital (20 mg/kg). Using a midline abdominal incision, the infra-renal aorta was isolated and the animals were randomized into three groups. Ischemia-reperfusion group (IRG, n = 5): rats that underwent 30 min of aorta occlusion and were followed during 120 min of reperfusion. Ischemia group (IG, n = 5): rats that underwent 30 min of aorta occlusion. Control group (CG, n = 5): rats that underwent sham operations without aorta occlusion and were followed during 150 min. Histopathologic examination of the right lung was performed. The pulmonary edema was studied by morphometric analysis and so was the leukocytes infiltration. Eighteen rats (6 rats in each group) were studied with respect to lung fresh/dry weight ratio. ANOVA was used to analyze the morphometric results and the lung fresh/dry weight ratio, with Bonferroni adjustment for paired multiple comparisons. Animals of the IRG presented more pulmonary edema than those of the IG and CG (0.24 vs. 0.17 and 0.17, p < 0.001). There was no difference between the groups regarding the lung fresh/dry weight ratio. There were more inflammatory cells in the lungs of the rats submitted to ischemia-reperfusion. The authors concluded that infra-renal abdominal aorta cross-clamping and unclamping are associated with pulmonary edema. This edema is not induced by elevation of the hydrostatic pressure due to the aortic occlusion, since it was not seen in animals that were only submitted to ischemia.

 


Keywords: ischemia, reperfusion, pulmonary edema, inbred strains rats

 


Respiratory muscles in interstitial lung disease: poorly explored and poorly understood

Musculatura respiratória em doença pulmonar intersticial: pouco explorada e pouco compreendida

Bruno Guedes Baldi1, João Marcos Salge1

J Bras Pneumol.2016;42(2):82-83

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Pulmonary and cutaneous nocardiosis in a patient treated with corticosteroids

Nocardiose pulmonar e cutânea em paciente usuário de corticosteróide

Bruno Guedes Baldi, Alfredo Nicodemos Cruz Santana, Teresa Yae Takagaki

J Bras Pneumol.2006;32(6):592-595

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Nocardiosis is a localized or disseminated infection caused by gram-positive bacteria of the genus Nocardia. The infection most commonly affects the lungs, skin and central nervous system. Nocardiosis principally occurs in individuals with cellular immunodeficiency and should be considered in the differential diagnosis when such individuals present respiratory, cutaneous or neurological alterations. Herein, we report a case of pulmonary and cutaneous nocardiosis in a patient receiving oral corticosteroids to treat bronchiolitis obliterans accompanied by organizing pneumonia of unknown origin. After long-term treatment with sulfamethoxazole-trimethoprim, the clinical and radiological profile improved.

 


Keywords: Nocardia infections; Lung diseases; Bronchiolitis obliterans; Adrenal cortex hormones; Case reports [Publication type]

 


New steps for the international consolidation of the Brazilian Journal of Pulmonology

Novos passos para a consolidação internacional do Jornal Brasileiro de Pneumologia

Carlos Roberto Ribeiro Carvalho, Bruno Guedes Baldi, Carlos Viana Poyares Jardim, Pedro Caruso, Rogério Souza

J Bras Pneumol.2014;40(4):325-326

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Eosinophilic pneumonia: remember topical drugs as a potential etiology

Pneumonia eosinofílica: lembre-se de medicamentos tópicos como possível etiologia

Olívia Meira Dias1,a, Ellen Caroline Toledo do Nascimento2,b, Rodrigo Caruso Chate3,c, Ronaldo Adib Kairalla1,d, Bruno Guedes Baldi1,e

J Bras Pneumol.2018;44(6):522-524

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Tracheal polyp

Pólipo traqueal

Bruno Guedes Baldi, Caio Júlio César dos Santos Fernandes, João Marcos Salge, Teresa Yae Takagaki

J Bras Pneumol.2007;33(5):616-620

Abstract PDF PT PDF EN Portuguese Text

Benign tracheal tumors are rare, recurrent papillomatosis being the most common. They often simulate obstructive pulmonary diseases, such as asthma and chronic obstructive pulmonary disease, and patients with benign tracheal tumors often undergo long-term treatment for such diseases, without any improvement, Therefore, these tumors should be included in the differential diagnosis in patients presenting tracheobronchial tree obstruction. This report describes the case of a patient with a tracheal polyp. The patient presented symptoms for three years, and the spirometry findings suggested intrathoracic obstruction. The patient presented complete clinical and spirometric recovery after bronchoscopic resection of the tumor.

 


Keywords: Polyps; Airway obstruction; Spirometry; Bronchoscopy.

 


Characterization and outcomes of pulmonary alveolar proteinosis in Brazil: a case series

Proteinose alveolar pulmonar: caracterização e desfechos em uma série de casos no Brasil

Rodolfo Augusto Bacelar de Athayde1,a, Fábio Eiji Arimura1,b, Ronaldo Adib Kairalla1,c, Carlos Roberto Ribeiro Carvalho1,d, Bruno Guedes Baldi1,e

J Bras Pneumol.2018;44(3):231-236

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Objective: Pulmonary alveolar proteinosis (PAP) is a rare disease, characterized by the alveolar accumulation of surfactant, which is composed of proteins and lipids. PAP is caused by a deficit of macrophage activity, for which the main treatment is whole-lung lavage (WLL). We report the experience at a referral center for PAP in Brazil. Methods: This was a retrospective study involving patients with PAP followed between 2002 and 2016. We analyzed information regarding clinical history, diagnostic methods, treatments, and outcomes, as well as data on lung function, survival, and complications. Results: We evaluated 12 patients (8 of whom were women). The mean age was 41 ± 15 years. Most of the patients were diagnosed by means of BAL and transbronchial biopsy. The mean number of WLLs performed per patient was 2.8 ± 2.5. One third of the patients never underwent WLL. Four patients (33.3%) had associated infections (cryptococcosis, in 2; nocardiosis, in 1; and tuberculosis, in 1), and 2 (16.6%) died: 1 due to lepidic adenocarcinoma and 1 due to complications during anesthesia prior to WLL. When we compared baseline data with those obtained at the end of the follow-up period, there were no significant differences in the functional data, although there was a trend toward an increase in SpO2. The median follow-up period was 45 months (range, 1-184 months). The 5-year survival rate was 82%. Conclusions: To our knowledge, this is the largest case series of patients with PAP ever conducted in Brazil. The survival rate was similar to that found at other centers. For symptomatic, hypoxemic patients, the treatment of choice is still WLL. Precautions should be taken in order to avoid complications, especially opportunistic infections.

 



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

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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.

 


Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations

Uso de sirolimo no tratamento de linfangioleiomiomatose: resposta favorável em pacientes com diferentes manifestações extrapulmonares

Carolina Salim Gonçalves Freitas1, Bruno Guedes Baldi2, Mariana Sponholz Araújo1, Glaucia Itamaro Heiden1, Ronaldo Adib Kairalla3, Carlos Roberto Ribeiro Carvalho4

J Bras Pneumol.2015;41(3):275-280

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Objective: Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day). Methods: We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus. Results: After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma. Conclusions: Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.

 


Keywords: Neoplasms; Lymphangioleiomyomatosis/therapy; TOR serine-threonine kinases; Sirolimus.

 


 

 


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