Brazilian Journal of Pulmonology

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

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Histological features and survival in idiopathic pulmonary fibrosis

Achados histológicos e sobrevida na fibrose pulmonar idiopática

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

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

Abstract PDF PT

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

 


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

 


Lung adenocarcinoma, dermatomyositis, and Lambert-Eaton myasthenic syndrome: a rare combination

Adenocarcinoma pulmonar, dermatomiosite e síndrome miastênica de Lambert-Eaton: uma rara associação

Fernanda Manente Milanez, Carlos Alberto de Castro Pereira, Pedro Henrique Duccini Mendes Trindade, Ricardo Milinavicius, Ester Nei Aparecida Martins Coletta

J Bras Pneumol.2008;34(5):333-336

Abstract PDF PT PDF EN Portuguese Text

The incidence of lung neoplasms is increasing in Brazil and in the world, probably as a result of the increase in smoking. Due to the greater number of cases, atypical presentations appear. We report the case of a 66-year-old hypertensive male smoker who presented progressive proximal muscular weakness and, in two months, evolved to dysphagia, dysphonia, and V-shaped skin lesions on the chest. A chest X-ray showed a spiculated pulmonary nodule in the right upper lobe. The biochemical analysis revealed elevated creatine kinase levels. After complementary tests and biopsies, the patient underwent right upper lobectomy. Histopathology showed a moderately differentiated adenocarcinoma. The overall analysis of the case and a review of the literature allow us to suggest that the clinical profile of the patient was a result of an overlap of two paraneoplastic syndromes (dermatomyositis and Lambert-Eaton myasthenic syndrome) secondary to lung adenocarcinoma.

 


Keywords: Lung neoplasms; Paraneoplastic syndromes; Dermatomyositis; Lambert-Eaton myasthenic syndrome; Adenocarcinoma.

 


Evaluation of the histological parameters in usual interstitial pneumonia (idiopathic pulmonary fibrosis)

Avaliação de parâmetros histológicos na pneumonia intersticial usual (fibrose pulmonar idiopática)

Rimarcs Gomes Ferreira, Ester Nei Aparecida Martins Coletta, Osvaldo Giannotti Filho

J Bras Pneumol.2000;26(6):279-285

Abstract PDF PT

Idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) is a progressive interstitia pulmonary disease of unknown etiology. Since Hamman's and Rich's (1944) reports, many studies have tried to find a histological marker for the correlation between prognosis and response to therapy. However, there are many doubts regarding pathogenesis. In addition, it is generally accepted that response to therapy is related to the relative degree of cellularity and fibrosis. The purpose of this study is to describe the results of inflammatory/exudative changes, fibrotic/reparative changes, and airway alterations, using a semi-quantitative method by independent evaluation of two pathologists, in 24 open lung biopsies with the diagnosis of idiopathic pulmonary fibrosis. Fourteen histological features were analyzed using the 0 to 5 scale for interstitial alterations and the 0 to 2 scale for the airway changes. There was significant interobserver agreement for all histological features with Kw (Kappa) variations between 0.47 and 0.92. There was significant disagreement only for septal inflammatory intensity analysis, suggesting that these features must be discussed by the pathologists. The semi-quantitative method assessment was effective.

 


Keywords: Idiopathic pulmonary fibrosis (IPF). Usual interstitial pneumonia (UIP). Semi-quantitative method assessment.

 


Bronchiolitis associated with exposure to artificial butter flavoring in workers at a cookie factory in Brazil

Bronquiolite associada à exposição a aroma artificial de manteiga em trabalhadores de uma fábrica de biscoitos no Brasil

Zaida do Rego Cavalcanti, Alfredo Pereira Leite de Albuquerque Filho, Carlos Alberto de Castro Pereira, Ester Nei Aparecida Martins Coletta

J Bras Pneumol.2012;38(3):395-399

Abstract PDF PT PDF EN Portuguese Text

Objective: To report the cases of four patients with bronchiolitis caused by exposure to artificial butter flavoring at a cookie factory in Brazil. Methods: We described the clinical, tomographic, and spirometric findings in the four patients, as well as the lung biopsy findings in one of the patients. Results: All four patients were young male nonsmokers and developed persistent airflow obstruction (reduced FEV1/FVC ratio and FEV1 at 25-44% of predicted) after 1-3 years of exposure to diacetyl, without the use of personal protective equipment, at a cookie factory. The HRCT findings were indicative of bronchiolitis. In one patient, the surgical lung biopsy revealed bronchiolitis obliterans accompanied by giant cells. Conclusions: Bronchiolitis resulting from exposure to artificial flavoring agents should be included in the differential diagnosis of airflow obstruction in workers in Brazil.

 


Keywords: Diacetyl; Flavoring agents; Bronchiolitis.

 


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.

 


Delayed diagnosis of sarcoidosis is common in Brazil

Diagnóstico tardio da sarcoidose é comum no Brasil

Mauri Monteiro Rodrigues, Ester Nei Aparecida Martins Coletta, Rimarcs Gomes Ferreira, Carlos Alberto de Castro Pereira

J Bras Pneumol.2013;39(5):539-546

Abstract PDF PT PDF EN Portuguese Text

Objective: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. Methods: We evaluated patients with a biopsy-proven diagnosis of sarcoidosis, using a questionnaire that addressed the following: time since symptom onset and since the first medical visit; and the number and specialty of the physicians visited. We divided the patients by the timeliness of the diagnosis-timely (< 6 months) and delayed ( 6 months)-comparing the two groups in terms of systemic and pulmonary symptoms; extrathoracic involvement; spirometric data; radiological staging; level of education; income; and tuberculosis (diagnosis and treatment). Results: We evaluated 100 patients. The median number of physicians consulted was 3 (range, 1-14). In 11 cases, sarcoidosis was diagnosed at the first visit. In 54, the first physician seen was a general practitioner. The diagnosis of sarcoidosis was timely in 41 patients and delayed in 59. The groups did not differ in terms of gender; race; type of health insurance; level of education; income; respiratory/systemic symptoms; extrathoracic involvement; and radiological staging. In the delayed diagnosis group, FVC was lower (80.3 ± 20.4% vs. 90.5 ± 17.1%; p = 0.010), as was FEV1 (77.3 ± 19.9% vs. 86.4 ± 19.5%; p = 0.024), misdiagnosis with and treatment for tuberculosis ( 3 months) also being more common (24% vs. 7%, p = 0.032, and 20% vs. 0%; p = 0.002, respectively). Conclusions: The diagnosis of sarcoidosis is often delayed, even when the imaging is suggestive of sarcoidosis. Delayed diagnosis is associated with impaired lung function at the time of diagnosis. Many sarcoidosis patients are misdiagnosed with and treated for tuberculosis.

 


Keywords: Sarcoidosis; Sarcoidosis, pulmonary/diagnosis; Tuberculosis.

 


Pleuroparenchymal fibroelastosis: report of two cases in Brazil

Fibroelastose pleuroparenquimatosa: relato de dois casos no Brasil

Paula Silva Gomes1, Christina Shiang2, Gilberto Szarf3, Ester Nei Aparecida Martins Coletta4,5, Carlos Alberto de Castro Pereira6

J Bras Pneumol.2017;43(1):72-75

Abstract PDF PT PDF EN Portuguese Text

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease. It can be idiopathic or associated with any one of various conditions. To our knowledge, this is the first report of two cases of PPFE in Brazil. Our first patient presented with pleural and subpleural fibrosis in the upper lobes; a spiculated nodule in the left upper lobe; and a mild reticular pattern in the lower lobes. Surgical lung biopsy revealed PPFE in the upper lobes, including the nodule, and unclassified interstitial pneumonia in the left lower lobe. Our second patient had a history of exposure to domestic birds, indicating a risk of hypersensitivity pneumonitis, and presented with advanced lung disease, predominantly in the upper lobes, together with subpleural fibrosis.That patient underwent lung transplantation. In the explant specimen, PPFE and granulomas were identified, suggesting hypersensitivity pneumonitis as an associated cause.

 


Keywords: Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/etiology; Alveolitis, extrinsic allergic.

 


Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia accompanied by airflow obstruction

Hiperplasia de células neuroendócrinas pulmonares difusas com obstrução ao fluxo aéreo

Ester Nei Aparecida Martins Coletta, Larissa Rêgo Voss, Mariana Silva Lima, Jaquelina Sonoe Ota Arakaki, Juvêncio Câmara, Carlos D'Andretta Neto, Carlos Alberto de Castro Pereira

J Bras Pneumol.2009;35(5):489-494

Abstract PDF PT PDF EN Portuguese Text

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with airflow obstruction is a rare form of lung injury. All of the reported cases have been diagnosed by surgical lung biopsy. Only three of the reported cases presented with diffuse interstitial lung opacities on HRCT scans. We report three additional cases of this entity. All of the patients were female and presented with mild-to-moderate airflow obstruction. In the first case, transbronchial biopsy and imaging data were sufficient to make the diagnosis. Although the HRCT scans of all three cases revealed a mosaic pattern, that of the third patient also revealed diffuse interstitial infiltrate. In extremely rare cases, HRCT findings can simulate those seen in other interstitial lung diseases.

 


Keywords: Neuroendocrine cells; Carcinoid tumor; Bronchiolitis obliterans.

 


Padrões histológicos dos processos infiltrativos difusos em colagenoses

Ester Nei Aparecida Martins Coletta

J Bras Pneumol.1997;23(4):197-207

PDF PT



Tracheobronchopathia osteochondroplastica

Traqueobroncopatia osteocondroplástica

Mara Graziele Maciel Silveira1, Maria Vera Cruz de Oliveira Castellano2, Clarice Emiko Fuzi2, Ester Nei Aparecida Martins Coletta2, Guilherme Nogueira Spinosa2

J Bras Pneumol.2017;43(2):151-153

Abstract PDF PT PDF EN Portuguese Text

Tracheobronchopathia osteochondroplastica is a rare benign disease, of unknown cause, characterized by numerous sessile, cartilaginous, or bony submucosal nodules distributed throughout the anterolateral walls, projecting into the laryngotracheobronchial lumen. In general, tracheobronchopathia osteochondroplastica is diagnosed incidentally during bronchoscopy or autopsy and is not associated with a specific disease. We report the case of a male patient who was diagnosed with tracheobronchopathia osteochondroplastica via bronchoscopy and biopsy.

 


Keywords: Dyspnea; Tracheal diseases; Bronchoscopy.

 


 

 


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