Navegando por Palavras-chave "Interstitial Lung Disease"
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- ItemAcesso aberto (Open Access)Biópsia transbrônquica em pneumonite de hipersensibilidade crônica(Universidade Federal de São Paulo (UNIFESP), 2019-06-27) Botelho, Andre Bezerra [UNIFESP]; Pereira, Carlos Alberto De Castro [UNIFESP]; http://lattes.cnpq.br/5439717016826243; Universidade Federal de São Paulo (UNIFESP)Introduction: The diagnosis of chronic hypersensitivity pneumonitis (cHP) is based on clinical data, tomographic patterns and, in several cases, on pathologic findings. There are some proposed diagnostic criteria, but none is widely accepted. The role of transbronchial biopsy (TBB) in HP is still uncertain. Aim: To analyze the yield of the TBB in patients with cHP according tomographic findings and to evaluate the importance of the bronchoalveolar lavage (BAL) in the diagnosis approach. Methods: Retrospective study of patients with cHP submitted to TBB in two specialized centers in interstitial lung diseases in São Paulo - Brazil, between 1999 and 2017. Diagnostic criteria according Salisbury et al were applied to diagnosis. The disease was classified as non-fibrotic or fibrotic according findings of fibrosis on HRCT. Findings in TBB were classified as definitive (granulomas and/or multinucleated giant’s cells, associated or not with peribronchiolar infiltration of lymphocytes and plasma cells and bronchiolocentric distribution) and compatible (indicative of small airway injury, such as peribronchiolar metaplasia, organizing pneumonia and intra-alveolar xanthomatous macrophages). Results: One hundred and nine patients were included, with female predominance (70.6%), mean age 61.4 years and mean FVC 72.1%. The main symptom was dyspnea, in 95 cases (87.1%). The tomographic finding more common was ground-glass, in 90 cases (82.6%), followed by any indicative findings of fibrosis, in 76 (69.7%) and mosaic attenuation/air trapping, in 67 (61.5%). The main identified exposures were to molds (60.6%) and birds (48.6%). The presence of definitive findings of HP were seen in 15 cases (13.8%) and compatible findings in 33 (30.2%), with total yield of 44%. Pathologic definite findings were more common in cases without fibrosis on HRCT (24,2% vs. 9,2%, p= 0.036), whereas the presence compatible findings were more common in fibrotic HP (38.1% vs. 12.1%, p= 0.007). The number of samples obtained in the TBB did not influences the results. 52 patients had differential cytology of the BAL. Lymphocytosis (> 20%) was present in 51.9% of the patients. The median of the percentage of lymphocytes in the BAL was 20,0% (interquartile interval: 11,5% to 29,5%). There wasn’t difference in the median of the lymphocytes according tomographic patterns. Conclusion: The TBB has a considerable yield to the diagnosis of HP and in association with BAL should be used as the initial option in the investigation of the disease.
- ItemSomente MetadadadosCaracterísticas clínicas, resposta ao tratamento e sobrevida em uma coorte de doenças pulmonares intersticiais com achados de miosites(Universidade Federal de São Paulo (UNIFESP), 2021) Miranda, Gustavo Frazatto Medeiros De [UNIFESP]; Pereira, Carlos Alberto De Castro [UNIFESP]; Universidade Federal de São PauloIntroduction: Interstitial lung diseases (ILD) include a large number of conditions, which can sometimes be associated with myositis findings. Objectives: The aims of present study were to compare the clinical, functional, and tomographic characteristics, treatment response and survival in a Brazilian cohort of patients with ILD and myositis, classified into two groups: (1) With myositis-specific antibodies (MSA); and (2) myositis-associated antibodies (MAA) or clinical features of myositis. Methods: The diagnosis of myositis was characterized by: (1) presence of a mechanic's hands; or (2) elevation of muscle enzymes (creatine phosphokinase or aldolase) 2.5 times above the upper limit of normal associated with subjective muscle weakness or myalgia. All patients included in the study had pulmonary involvement on high-resolution chest tomography (HRCT). Data between the two groups were compared by t-test, median test, chi-square test, Fisher's exact test and survival curves were compared by Kaplan-Meier test. Significant functional decline was characterized by decrease in forced vital capacity (FVC) ≥ 5% of predicted value. Results: Mean age was 51 years and median follow-up was 50 months [IQ=25 - 98]. The mean FVC was 61.9 ± 16.6%. Among 77 patients analyzed, 43 (55.9%) were included in group 1, and 34 (44.1%) in group 2. The groups were similar, except that group 2 had more male subjects (61% versus 39%), more commonly audible pulmonary crackles (91% versus 72) and no SD pattern in capillaroscopy. There was no difference in types and duration of treatment between groups, neither in survival. Overall survival was greater than 80% at five years. Predictive factors for shorter survival were the presence honeycombing on HRCT (log-rank=34.65; p<0.001), and a decrease in FVC from 5% of predicted in longitudinal assessment (log-rank=16.50; p<0.001). Conclusion: Clinical, functional, and tomographic characteristics are similar between patients with ILD and presence of specific antisynthetase antibodies, and those with clinical findings of myositis, with or without associated antibodies. Presence of honeycombing on HRCT and a decrease in predicted FVC ≥ 5% are predictors of lower survival.
- ItemSomente MetadadadosEscore Multidimensional Simples Para Predição Do Curso Clínico Da Sarcoidose Na Avaliação Inicial(Universidade Federal de São Paulo (UNIFESP), 2017-11-30) Castro, Marina Dornfeld Cunha [UNIFESP]; Pereira, Carlos Alberto De Castro [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Sarcoidosis is a systemic granulomatous disease of unknown etiology. The outcome is variable and is mainly related to persistent inflammation process and development of fibrosis. Prognostic features have been described, but there is no validated model to predict the evolution of the disease. Aim: To find predictive features associated to clinical course of sarcoidosis at initial evaluation and develop a predictive score. Methods: This was a retrospective study of a cohort of patients with diagnosis of sarcoidosis, followed in reference centers involved in interstitial lung disease. Clinical, radiographic and functional data were compared by univariate analysis between patients with limited course – spontaneous involution or stability after a course of therapy and persistent – relapse or resistance to treatment, persistent treatment or chronic stable disease. Data related to persistent disease were selected by multivariate analysis and a prognostic score was developed.Results: 200 patients were included. 63% were female. Mean age at diagnosis was 50 years old. Median of time of symptoms was 12 months. Early diagnosis was characterized by time of symptoms ≤ 12 months and was found in 57% of cases. Dyspnea and cough were the most common pulmonary symptoms. Treatment for tuberculosis was prescribed before diagnosis in 44 patients. The most common radiologic stage was II (lung parenchyma and thoracic adenopathy). Mean forced vital capacity (FVC) was 84.9 ± 18.8% of predicted, with 37% of the cases with FVC below lower limit of reference. Relevant systemic involvement was detected in 37% of the patients. During the evolution, 77% of the cases needed immunosuppressive treatment and the course of disease was classified as persistent in 115 cases. By logistic regression, excluding 40 patients with pulmonary fibrosis, the findings related to persistent disease were: parenchymal lung involvement [odds ratio (OR) 3.83], late diagnosis (OR 2.82), dyspnea (OR 2.80), systemic involvement (OR 2.55) and reduced FVC (OR 2.33). A score was developed, taking into account the weight of the variable (1 point each), with positive predictive value for persistent disease in A score (≤ 1 point) and C score (≥ 4 points) of 17% and 88% respectively. Conclusion: A predictive score for sarcoidosis outcome can be derived by multiple variables at initial evaluation, allowing prediction of the clinical course of disease in a significant number of cases. The results should be confirmed in a validation cohort.