Publications (22)26.66 Total impact
- [Show abstract] [Hide abstract] ABSTRACT: Prediction models for survival at baseline evaluation have been proposed in IPF but some are difficult to use. The aim of present study was to develop a new mortality risk scoring system for patients with IPF at initial evaluation. One hundred twenty with definitive IPF were selected through a review of standardized medical records for interstitial lung diseases. Patients with resting SpO2<89% were excluded. Significant individual predictors we derived by a Cox proportional hazards model and transformed in categorical data according to cut-off points. Beta coefficients for each predictor were similar, so a score was created considering the sum of dichotomic (0 or 1) transformed variables. Median follow-up time was 37.5 months. At the end of follow-up, 80 patients had died. Independent predictors of mortality by multivariate analysis included dyspnea (at rest or to light or moderate activities), FVC<70%,FEV1/FVC>0.89 and DLCO ≤ 40%. Resting SpO2 and ExSpO2 were excluded in final analysis. The hazard ratios ranged from 1.95 for dyspnoea to 2.30 for DLCO. When the total score was 0 (Stage I, n=28), median survival time was 68 months; when 1 or 2 (Stage II, n=69), it was 45 months; and when 3 or 4 (Stage III, n=23), it was 19 months (log rank= 60.44, p<0.001). The score can separate IPF patients with high, intermediate and low survival. (Sarcoidosis Vasc Diffuse Lung Dis 2015; 32: 121-128).
- [Show abstract] [Hide abstract] ABSTRACT: Objective: To evaluate the value of differential cytology of bronchoalveolar lavage (BAL) in common interstitial lung diseases (ILDs) and its relation with high-resolution computed tomography (HRCT) fibrosis. Study design: A retrospective analysis of BAL from 91 patients. The total and percentage of cells were collected and analyzed according to published guidelines. Results: The main diagnoses were connective tissue diseases (n = 24), hypersensitivity pneumonitis (HP) (n = 15), sarcoidosis (n = 12), idiopathic pulmonary fibrosis (IPF) (n = 14), smoking-related interstitial lung diseases (sr-ILDs) (n = 8), aspiration (n = 8), and others (n = 10). The lymphocyte percentages differed among diagnoses (F = 2.85, p = 0.008). A greater number of lymphocytes were found in sarcoidosis (Md = 20%, IQR = 12-45%), HP (Md = 29%, IQR = 13-55%), and organizing pneumonia (Md = 28, IQR = 9-50%). No patient with IPF presented with > 25% lymphocytes in BAL. In 6/15 (40%) of the HP cases, the percentage of lymphocytes was < 25%. A total of 56 participants (62%) displayed HRCT findings that were indicative of fibrosis. In those cases the percentage of lymphocytes was 10.1 (IQR = 6.1-18.0) as compared with 20.2% (IQR = 9.7-37.5) of those 35 cases without fibrosis (p = 0.007). In patients with fibrosis in HRCT, the percentage of neutrophils was 11.3 (IQR = 4.1-21.4) as compared with 3.8 (IQR = 1.4-3.8) in those without fibrosis (p = 0.009). Conclusion: Several patterns in differential cytology are observed in common ILDs. The presence of fibrosis has an impact on these findings.
- [Show abstract] [Hide abstract] ABSTRACT: To determine the frequency of and the factors related to delayed diagnosis of sarcoidosis in Brazil. 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). 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). 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.
- [Show abstract] [Hide abstract] ABSTRACT: This report presents a case of cardiac sarcoidosis initially manifested with atrial fibrillation. This behavior is very uncommon in spite of the fact that the disease is multisystemic, affecting predominantly the lungs. It is emphasized that the diagnosis of the cardiac involvement is difficult, and when this occurs, can lead to conducting system disturbances, heart failure or sudden death (SD). The diagnosis can be made by evaluating the clinical manifestations, the noninvasive tests like ECG, Holter monitoring, chest radiography, thoracic computed tomography, magnetic resonance image and positron emission tomography. In general, sarcoidosis is treated with steroid compounds with good outcome, mainly when performed in the initial phases of the disease. Other cardiac manifestations, such as arrhythmias, atrioventricular block or heart failure, are managed similar to other cardiomyopathies.
- [Show abstract] [Hide abstract] ABSTRACT: 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.
- [Show abstract] [Hide abstract] ABSTRACT: 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.
- [Show abstract] [Hide abstract] ABSTRACT: To report the cases of four patients with bronchiolitis caused by exposure to artificial butter flavoring at a cookie factory in Brazil. We described the clinical, tomographic, and spirometric findings in the four patients, as well as the lung biopsy findings in one of the patients. 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. Bronchiolitis resulting from exposure to artificial flavoring agents should be included in the differential diagnosis of airflow obstruction in workers in Brazil.
Conference Paper: Organizing Pneumonia - Analysis Of 145 Cases
- [Show abstract] [Hide abstract] ABSTRACT: A definite cause of sarcoidosis has not been identified, however past research suggests that environmental factors may be triggers of the granulomatous response in genetically susceptible individuals. A 22-year-old male non-smoker, presented with progressive exertional dyspnea and cough of 3 months duration. One year before, when he started working in tunnel excavation, he had a normal chest radiograph. Chest imaging revealed bilateral nodules and masses of peribronchovascular distribution plus mediastinal lymphadenomegaly. Histologic lymph node analysis revealed non-caseating confluent granulomas. Sarcoidosis was diagnosed. The patient was treated with corticosteroids and advised to change jobs. Complete remission of the disease was achieved and persisted for at least one year without steroid treatment. Sarcoidosis is believed to have environmental triggers. The timing of the onset of sarcoidosis in this patient following intensive exposure to tunnel dust suggests an environmental contribution. The recognition that sarcoidosis may have occupational triggers have medical, employment, and legal implications.
- [Show abstract] [Hide abstract] ABSTRACT: In sarcoidosis, clinical presentations and outcomes vary widely. To characterize the clinical phenotypes of sarcoidosis, by factor analysis, in a series of cases with long-term follow-up. We conducted a retrospective study involving 137 patients with biopsy-confirmed sarcoidosis, recruited from two referral centers in São Paulo, Brazil. Organ involvement was evaluated in accordance with a previously established protocol. Sarcoidosis phenotypes were characterized by factor analysis. Follow-up ranged from 6 to 144 months. Four factors (phenotypes) were identified: relevant residual pulmonary fibrosis; relapse; residual airflow limitation; and acute disease. The four factors collectively accounted for 66% of the total variance. Patients with relevant residual pulmonary fibrosis were older and presented with the following: greater symptom duration; skin involvement; low forced vital capacity; low forced expiratory volume in one second/forced vital capacity ratio; and more advanced radiographic stages at baseline. The relapse phenotype was associated with chronic disease, greater dyspnea severity, neurologic involvement, and cardiac involvement. Patients with residual airflow limitation more often had airflow obstruction at baseline, chronic disease, and relevant residual pulmonary fibrosis. Acute disease was associated with being younger, weight loss, scoring lower for dyspnea, and having extensive involvement. Abnormal calcium metabolism was associated with acute disease and with relapse. Sarcoidosis can be categorized into four different clinical phenotypes: three that are chronic; and one that is acute and self-limiting. In many cases, these phenotypes can be easily recognized.
- [Show abstract] [Hide abstract] ABSTRACT: 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.
- [Show abstract] [Hide abstract] ABSTRACT: A hiperplasia de células neuroendócrinas pulmonares difusas com obstrução ao fluxo aéreo é uma lesão pulmonar rara. Todos os casos publicados foram diagnosticados por biópsia pulmonar cirúrgica. Apenas três casos relatados apresentavam opacidades intersticiais difusas na TCAR. Nós relatamos três casos adicionais desta entidade. Todos eram mulheres, com obstrução leve ou moderada ao fluxo aéreo. No primeiro caso, uma biópsia transbrônquica, associada com dados de imagem foram considerados suficientes para o diagnóstico. Um padrão em mosaico foi observado nos três casos, mas no terceiro um infiltrado pulmonar difuso foi também observado. Em casos muito raros, o aspecto na TCAR pode simular aquele encontrado em outras doenças pulmonares intersticiais.
- [Show abstract] [Hide abstract] ABSTRACT: In hypersensitivity pneumonitis (HP), survival can be predicted on the basis of the severity of fibrosis in surgical lung biopsy, but few data are available on the influence of clinical, functional, tomographic and histologic findings on prognosis. To describe the impact on survival of clinical data, histological patterns, and HRCT findings in subacute/chronic HP. A retrospective analysis of 103 patients diagnosed with HP submitted to surgical lung biopsy. Chronic HP was characterized by HRCT findings indicative of fibrosis (n=76). The most relevant exposures were to molds and birds. Lung biopsies revealed typical HP with granulomas in 46 patients, bronchiolocentric interstitial pneumonia in 27, and non-specific interstitial pneumonia (NSIP) in 16. By univariate analysis, several findings were predictors of mortality: older age, male sex, velcro crackles, higher FEV(1)/FVC ratio, lower oxygen saturation during exercise, and absence of mosaic pattern/air trapping and presence of fibrosis on HRCT. By multivariate analysis, remained significant: age (p=0.007), oxygen saturation during exercise (p=0.003), and mosaic pattern/air trapping on HRCT (p=0.004). Patients with NSIP had a greater survival than did those with typical histology and those with bronchiolocentric pneumonia (p=0.033). A wide range of histological features are found in HP. Typical findings are seen in 45% of cases. Other common patterns are NSIP and centriacinar lesions. Survival is better in patients with NSIP and worse in those with older age, desaturation during exercise, and absence of mosaic pattern/air trapping on HRCT.
- [Show abstract] [Hide abstract] ABSTRACT: 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.
- [Show abstract] [Hide abstract] ABSTRACT: PaO2 during exertion is a significant predictor of IPF survival. The aim of study was to assess oxygen desaturation for predicting survival in IPF patients at the end of a 4-minute step test. A longitudinal study was done in 59 patients with IPF from February 1998 to January 2005. Upon initial examination, lung function testing was performed, as were 4-minute step tests, in which patients stepped up and down on a 20-cm single step at a self-paced rate. In the final minute, oxygen saturation by pulse oximetry (SpO2) was measured. Median survival was 58 months. SpO2 at rest and during stepping, dyspnea score, as well as FVC% and DLCO% were found to be significant predictors of mortality. Desaturation to 89% or less,correlated with a hazard ratio of 2.39 for IPF mortality (95% CI, 1.16-3.63; p < 0.0001). In patients with such desaturation, four-year survival was 39%, compared to 96% in those with no desaturation. In a multivariate Cox analysis, only SpO2 during stepping remained significant (p < 0.0001). A DLco < 45% of predicted (n=40 patients) correlated with a hazard ratio of 2.23 for mortality (95% CI, 0.73-3.71; p < 0.0001). When the analysis was repeated including DLco, SpO2 and DLco remained significant (p < 0.05). Desaturation to 89% or less in a 4-min step test is a strong predictor of mortality in IPF patients.
Universidade Federal de São Paulo
San Paulo, São Paulo, Brazil
- School of Medicine