Brazilian Journal of Pulmonology

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

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Pulmonary involvement in Behcet's disease: a positive single-center experience with the use of immunosuppressive therapy

Acometimento pulmonar na doença de Behçet: uma boa experiência com o uso de imunossupressores

Alfredo Nicodemos Cruz Santana, Telma Antunes, Juliana Monteiro de Barros, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro de Carvalho, Carmen Silvia Valente Barbas

J Bras Pneumol.2008;34(6):362-366

Abstract PDF PT PDF EN Portuguese Text

Objective: Behcet's syndrome, or Behcet's disease (BD), is a multisystem pathology, and survival is related to pulmonary involvement. However, it appears that different treatments correlate with different prognoses. The aim of this study was to evaluate clinical and tomographic evolution, as well as the survival, of patients with BD-related pulmonary involvement. Methods: A retrospective review of our experience with pulmonary manifestations in patients with BD treated at our institution between January 1, 1988 and April 30, 2006. The clinical, radiological, treatment and survival data were obtained from medical charts. Results: We identified 9 patients with BD-related pulmonary involvement. The mean age was 34 ± 11.5 years, and 7 of the patients were male. The radiological findings were as follows: pulmonary artery aneurysm (PAA) in 8 patients; pulmonary embolism in 3 (translating to an incidence of 5.11 cases/100 patient-years); alveolar hemorrhage in one; and pulmonary hypertension in one. The treatment consisted of immunosuppression with prednisone plus chlorambucil (or cyclophosphamide or mycophenolate mofetil) in all patients, with partial or complete resolution of the PAAs. One patient with a PAA and pulmonary hypertension also received sildenafil and warfarin, with good clinical and tomographic response (the first report in the English literature). In our sample, the mean duration of the follow-up period was 6.52 years. The three-year survival rate was 88.8%, as was the five-year survival rate. Conclusions: Patients with BD-related pulmonary involvement can present good survival with immunosuppressive therapy, and BD should be borne in mind as a possible cause of pulmonary hypertension and alveolar hemorrhage.

 


Keywords: Behcet Syndrome; Lung diseases, interstitial; Pulmonary circulation; Hypertension, pulmonary; Pulmonary embolism; Alveolar.

 


Takotsubo cardiomyopathy triggered by 2 adrenergic agonist

Cardiomiopatia de takotsubo desencadeada pelo uso de agonista β2-adrenérgico

Vera Maria Cury Salemi, Edmar Atik, Ronaldo Adib Kairalla, Eduardo Lira Queiroz, Leonardo Vieira da Rosa, Roberto Kalil Filho

J Bras Pneumol.2011;37(4):560-562

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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

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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

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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.

 


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.

 


Interstitial lung disease in scleroderma

Doença intersticial pulmonar na esclerodermia

Ronaldo Adib Kairalla

J Bras Pneumol.2005;31(4):1-3

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Pulmonary involvement in rheumatoid arthritis: evaluation by radiography and spirometry

Doença pulmonar em pacientes com artrite reumatoide: avaliação radiográfica e espirométrica

Alexandre Melo Kawassaki1, Daniel Antunes Silva Pereira1, Fernando Uliana Kay2, Ieda Maria Magalhães Laurindo3, Carlos Roberto Ribeiro Carvalho4, Ronaldo Adib Kairalla1

J Bras Pneumol.2015;41(4):331-342

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Objective: To determine whether simple diagnostic methods can yield relevant disease information in patients with rheumatoid arthritis (RA). Methods: Patients with RA were randomly selected for inclusion in a cross-sectional study involving clinical evaluation of pulmonary function, including pulse oximetry (determination of SpO2, at rest), chest X-ray, and spirometry. Results: A total of 246 RA patients underwent complete assessments. Half of the patients in our sample reported a history of smoking. Spirometry was abnormal in 30% of the patients; the chest X-ray was abnormal in 45%; and the SpO2 was abnormal in 13%. Normal chest X-ray, spirometry, and SpO2 were observed simultaneously in only 41% of the RA patients. A history of smoking was associated with abnormal spirometry findings, including evidence of obstructive or restrictive lung disease, and with abnormal chest X-ray findings, as well as with an interstitial pattern on the chest X-ray. Comparing the patients in whom all test results were normal (n = 101) with those in whom abnormal test results were obtained (n = 145), we found a statistically significant difference between the two groups, in terms of age and smoking status. Notably, there were signs of airway disease in nearly half of the patients with minimal or no history of tobacco smoke exposure. Conclusions: Pulmonary involvement in RA can be identified through the use of a combination of diagnostic methods that are simple, safe, and inexpensive. Our results lead us to suggest that RA patients with signs of lung involvement should be screened for lung abnormalities, even if presenting with no respiratory symptoms.

 


Keywords: Arthritis, rheumatoid; Lung diseases, interstitial; Spirometry; Radiography, thoracic; Airway ob-struction.

 


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

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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.

 


Hard metal lung disease: a case series

Doença pulmonar por metal duro: uma série de casos

Rafael Futoshi Mizutani1, Mário Terra-Filho1,2, Evelise Lima1, Carolina Salim Gonçalves Freitas1, Rodrigo Caruso Chate3, Ronaldo Adib Kairalla1,2, Regiani Carvalho-Oliveira4, Ubiratan Paula Santos1

J Bras Pneumol.2016;42(6):447-452

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Objective: To describe diagnostic and treatment aspects of hard metal lung disease (HMLD) and to review the current literature on the topic. Methods: This was a retrospective study based on the medical records of patients treated at the Occupational Respiratory Diseases Clinic of the Instituto do Coração, in the city of São Paulo, Brazil, between 2010 and 2013. Results: Of 320 patients treated during the study period, 5 (1.56%) were diagnosed with HMLD. All of those 5 patients were male (mean age, 42.0 ± 13.6 years; mean duration of exposure to hard metals, 11.4 ± 8.0 years). Occupational histories were taken, after which the patients underwent clinical evaluation, chest HRCT, pulmonary function tests, bronchoscopy, BAL, and lung biopsy. Restrictive lung disease was found in all subjects. The most common chest HRCT finding was ground glass opacities (in 80%). In 4 patients, BALF revealed multinucleated giant cells. In 3 patients, lung biopsy revealed giant cell interstitial pneumonia. One patient was diagnosed with desquamative interstitial pneumonia associated with cellular bronchiolitis, and another was diagnosed with a hypersensitivity pneumonitis pattern. All patients were withdrawn from exposure and treated with corticosteroid. Clinical improvement occurred in 2 patients, whereas the disease progressed in 3. Conclusions: Although HMLD is a rare entity, it should always be included in the differential diagnosis of respiratory dysfunction in workers with a high occupational risk of exposure to hard metal particles. A relevant history (clinical and occupational) accompanied by chest HRCT and BAL findings suggestive of the disease might be sufficient for the diagnosis.

 


Keywords: Lung diseases, interstitial; Cobalt; Tungsten; Occupational exposure; Hard metal.

 


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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Immunophenotyping and extracellular matrix remodeling in pulmonary and extrapulmonary sarcoidosis

Imunofenotipagem e remodelamento da matriz extracelular na sarcoidose pulmonar e extrapulmonar

Pedro Henrique Ramos Quintino da Silva, Edwin Roger Parra, William Sanches Zocolaro, Ivy Narde, Fabíola Rodrigues, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro de Carvalho, Vera Luiza Capelozzi

J Bras Pneumol.2012;38(3):321-330

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Objective: To investigate the significance of cellular immune markers, as well as that of collagen and elastic components of the extracellular matrix, within granulomatous structures in biopsies of patients with pulmonary or extrapulmonary sarcoidosis. Methods: We carried out qualitative and quantitative evaluations of inflammatory cells, collagen fibers, and elastic fibers in granulomatous structures in surgical biopsies of 40 patients with pulmonary and extrapulmonary sarcoidosis using histomorphometry, immunohistochemistry, picrosirius red staining, and Weigert's resorcin-fuchsin staining. Results: The extrapulmonary tissue biopsies presented significantly higher densities of lymphocytes, macrophages, and neutrophils than did the lung tissue biopsies. Pulmonary granulomas showed a significantly higher number of collagen fibers and a lower density of elastic fibers than did extrapulmonary granulomas. The amount of macrophages in the lung samples correlated with FVC (p < 0.05), whereas the amount of CD3+, CD4+, and CD8+ lymphocytes correlated with the FEV1/FVC ratio and VC. There were inverse correlations between TLC and the CD1a+ cell count (p < 0.05), as well as between DLCO and collagen/elastic fiber density (r = −0.90; p = 0.04). Conclusions: Immunophenotyping and remodeling both showed differences between pulmonary and extrapulmonary sarcoidosis in terms of the characteristics of the biopsy samples. These differences correlated with the clinical and spirometric data obtained for the patients, suggesting that two different pathways are involved in the mechanism of antigen clearance, which was more effective in the lungs and lymph nodes.

 


Keywords: Sarcoidosis; Granulomatous disease, chronic; Extracellular matrix; Immunophenotyping; Respiratory function tests.

 


Lymphadenopathy and fever in a chef during a stay in Europe

Linfadenomegalia e febre em chefe de cozinha durante viagem à Europa

Letícia Kawano-Dourado, Daniel Antunes Silva Peirera, Alexandre de Melo Kawassaki, Marisa Dolhnikoff, Marcos Vinicius da Silva, Ronaldo Adib Kairalla

J Bras Pneumol.2015;41(2):191-195

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This case illustrates a rare presentation (as lymphadenopathy and fever) of one of the most common zoonotic diseases worldwide-brucellosis-in a 22-year-old Brazilian male (a chef) who had recently returned to Brazil after having lived in and traveled around Europe for one year. The histopathology, clinical history, and response to treatment were all consistent with a diagnosis of brucellosis, which was confirmed by PCR in a urine sample. We also review some aspects of brucellosis, such as the clinical features, diagnosis, and management.

 


Keywords: Brucellosis; Fever; Lymph nodes; Brucella; Mononuclear phagocyte system; Granuloma.

 


Epipericardial fat necrosis: an unusual cause of chest pain

Necrose da gordura epipericárdica: uma causa incomum de dor torácica

Karina de Souza Giassi, André Nathan Costa, André Apanavicius, Guilherme Hipólito Bachion, Rafael Silva Musolino, Ronaldo Adib Kairalla

J Bras Pneumol.2013;39(5):627-629

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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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Usual interstitial pneumonia: a pattern or a disease? A reflection upon the topic

Pneumonia intersticial usual: um padrão ou uma doença? Reflexão sobre o assunto

Leticia Kawano-Dourado, Ronaldo Adib Kairalla

J Bras Pneumol.2013;39(1):111-112

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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.

 



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.

 


Tiotropium use and pulmonary function in patients with constrictive bronchiolitis

Uso de tiotrópio e função pulmonar em portadores de bronquiolite constritiva

Alexandre Melo Kawassaki, Letícia Kawano-Dourado, Ronaldo Adib Kairalla

J Bras Pneumol.2014;40(1):86-88

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