Fibrose pulmonar idiopática e outras doenças pulmonares intersticiais em pacientes com padrão tomográfico de pneumonia intersticial usual possível submetidos à biópsia pulmonar cirúrgica no Brasil
Arquivos
Data
2017-12-20
Autores
Tibana, Regina Celia Carlos 
Orientadores
Pereira, Carlos Alberto de Castro
Tipo
Tese de doutorado
Título da Revista
ISSN da Revista
Título de Volume
Resumo
Objetivo: determinar a frequência de fibrose pulmonar idiopática e outras doenças pulmonares intersticiais em pacientes com padrão tomográfico de pneumonia intersticial usual possível submetidos à biópsia pulmonar cirúrgica no Brasil. Métodos: casos com tomografia computadorizada de tórax com padrão de pneumonia intersticial usual possível e submetidos à biópsia pulmonar cirúrgica foram selecionados retrospectivamente de sete centros de doenças pulmonares intersticiais no Brasil. Todas as imagens de tomografia foram revistas por um radiologista e um pneumologista e classificadas por consenso, sem conhecimento dos dados clínicos e histológicos. Os casos de pneumonia intersticial usual definitvo e inconsistente foram excluídos após a revisão. O material histológico foi revisado por dois patologistas com experiência em doença pulmonar intersticial. O diagnóstico clínico final foi realizado por discussão multidisciplinar. Resultados: foram incluídos 61 casos, 67% do sexo masculino, média de idade de 65,2 ± 7 anos. Tabagismo foi descrito em 46,6% e exposição ambiental para pneumonia de hipersensibilidade em 33 de 61 casos (54%). Doença do refluxo gastroesofágico foi confirmada em 18 pacientes. Em dois terços os achados na tomografia computadorizada de tórax predominaram em campos pulmonares inferiores. Nos demais não foi observado predomínio em relação a campos pulmonares superiores e inferiores. Fibrose bronquiolocêntrica foi o padrão histológico mais frequente (55,7%; IC 95%: 42,4-68,5%), seguido de pneumonia intersticial usual (26,2%; IC 95%: 15,8-39,1%). Focos fibroblásticos foram observados em 59% dos casos (IC 95%: 45,7-71,4%). O diagnóstico mais frequente foi pneumonia de hipersensibilidade (37,7%; IC 95%: 25,6-51,0%), seguido de fibrose pulmonar idiopática (23,0%; IC 95%: 13,2-35,5%) e doença pulmonar intersticial secundária à doença do refluxo gastroesofágico (13,1%; IC 95%: 5,8-24,2%). Ausência de exposição ambiental associou-se ao diagnóstico de fibrose pulmonar idiopática (p= 0,001) e fibrose pulmonar idiopática ocorreu em 43% (IC 95%: 24,5-62,8%) dos 28 casos sem exposição ambiental. Entre os casos com exposição, fibrose pulmonar idiopática foi diagnosticada em dois (6,1%; IC 95%: 0,7-20,2%; p= 0,002). A avaliação de idade, sexo e escore de bronquiolectasias de tração não alterou os resultados. Conclusão: pneumonia de hipersensibilidade é a doença pulmonar intersticial mais frequente no padrão tomográfico de pneumonia intersticial usual possível no Brasil, seguida de fibrose pulmonar idiopática e doença pulmonar intersticial secundária à doença do refluxo gastroesofágico. Em pacientes sem exposição ambiental, a frequência de pneumonia intersticial usual e fibrose pulmonar idiopática não são suficientes para o diagnóstico sem biópsia pulmonar cirúrgica.
Objective: determine the frequency of idiopathic pulmonary fibrosis and other interstitial lung diseases in patients with possible usual interstitial pneumonia pattern on high-resolution computed tomography submitted to surgical lung biopsies in Brazil. Methods: patients with biopsy-proven interstitial lung disease and possible usual interstitial pneumonia pattern on high-resolution computed tomography scans were retrospectively identified from seven interstitial lung diseases centers in Brazil. All scans were reviewed by consensus by a clinician and a radiologist with large experience in interstitial lung diseases, blinded to histopathology results and clinical information. After review, cases with definitive usual interstitial pneumonia and inconsistent usual interstitial pneumonia pattern were excluded. All patients were submitted to surgical lung biopsies, that were reviewed and interpreted by two expert pulmonary pathologists. Final clinical diagnoses were done by a multidisciplinary team discussion. Results: sixty-one patients, 67% males, mean age 65,2 ± 7 years-old, 47% smokers / ex-smokers, were included. Exposure to possible causative antigens for hypersensitivity pneumonitis was found in 33 of the 61 cases (54%). Gastroesophageal reflux was confirmed in 18 cases. In two-thirds the findings on high-resolution computed tomography were predominant in lower lobes. In the remaining cases the distribution was diffuse. Bronchiolocentric fibrosis was the most common histological pattern (55.7%; 95% CI: 42.4-68.5%), followed by usual interstitial pneumonia (26.2%; 95% CI; 15.8-39.1%). Fibroblastic foci were observed in 59% of total cases (95% CI: 47.7-71.4%). The most common final diagnosis was hypersensitivity pneumonitis (37.7%; 95% CI 25.6-51.0%), followed by idiopathic pulmonary fibrosis (23%; 95%CI: 13.2-35.5%) and fibrosis secondary to gastroesophageal reflux (13.1%; 95% CI: 5.8-24.2%). Absence of environmental exposure, was associated with final diagnosis of idiopathic pulmonary fibrosis (p= 0.001), and idiopathic pulmonary fibrosis was the final diagnosis in 43% (95% CI: 24.5-62.8%) of 28 patients without exposure to possible hypersensitivity pneumonitis. In two cases with exposure (6.1%; 95% CI: 0.7-20.2%), idiopathic pulmonary fibrosis was the final diagnosis, p= 0.002. The addition of age, sex and a score for traction bronchiectasis did not change the results. Conclusion: the most common disease associated with possible usual interstitial pneumonia pattern on computed tomography is hypersensitivity pneumonitis, followed by idiopathic pulmonary fibrosis and fibrosis secondary to gastroesophageal reflux. In patients without environmental exposure, the frequency of usual interstitial pneumonia and a final diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to avoid the indication of surgical lung biopsy.
Objective: determine the frequency of idiopathic pulmonary fibrosis and other interstitial lung diseases in patients with possible usual interstitial pneumonia pattern on high-resolution computed tomography submitted to surgical lung biopsies in Brazil. Methods: patients with biopsy-proven interstitial lung disease and possible usual interstitial pneumonia pattern on high-resolution computed tomography scans were retrospectively identified from seven interstitial lung diseases centers in Brazil. All scans were reviewed by consensus by a clinician and a radiologist with large experience in interstitial lung diseases, blinded to histopathology results and clinical information. After review, cases with definitive usual interstitial pneumonia and inconsistent usual interstitial pneumonia pattern were excluded. All patients were submitted to surgical lung biopsies, that were reviewed and interpreted by two expert pulmonary pathologists. Final clinical diagnoses were done by a multidisciplinary team discussion. Results: sixty-one patients, 67% males, mean age 65,2 ± 7 years-old, 47% smokers / ex-smokers, were included. Exposure to possible causative antigens for hypersensitivity pneumonitis was found in 33 of the 61 cases (54%). Gastroesophageal reflux was confirmed in 18 cases. In two-thirds the findings on high-resolution computed tomography were predominant in lower lobes. In the remaining cases the distribution was diffuse. Bronchiolocentric fibrosis was the most common histological pattern (55.7%; 95% CI: 42.4-68.5%), followed by usual interstitial pneumonia (26.2%; 95% CI; 15.8-39.1%). Fibroblastic foci were observed in 59% of total cases (95% CI: 47.7-71.4%). The most common final diagnosis was hypersensitivity pneumonitis (37.7%; 95% CI 25.6-51.0%), followed by idiopathic pulmonary fibrosis (23%; 95%CI: 13.2-35.5%) and fibrosis secondary to gastroesophageal reflux (13.1%; 95% CI: 5.8-24.2%). Absence of environmental exposure, was associated with final diagnosis of idiopathic pulmonary fibrosis (p= 0.001), and idiopathic pulmonary fibrosis was the final diagnosis in 43% (95% CI: 24.5-62.8%) of 28 patients without exposure to possible hypersensitivity pneumonitis. In two cases with exposure (6.1%; 95% CI: 0.7-20.2%), idiopathic pulmonary fibrosis was the final diagnosis, p= 0.002. The addition of age, sex and a score for traction bronchiectasis did not change the results. Conclusion: the most common disease associated with possible usual interstitial pneumonia pattern on computed tomography is hypersensitivity pneumonitis, followed by idiopathic pulmonary fibrosis and fibrosis secondary to gastroesophageal reflux. In patients without environmental exposure, the frequency of usual interstitial pneumonia and a final diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to avoid the indication of surgical lung biopsy.
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Citação
TIBANA, Regina Celia Carlos. Fibrose pulmonar idiopática e outras doenças pulmonares intersticiais em pacientes com padrão tomográfico de pneumonia intersticial usual possível submetidos à biópsia pulmonar cirúrgica no Brasil. São Paulo, 2017. [133] p. Tese (Doutorado em Medicina: pneumologia) - Escola Paulista de Medicina (EPM), Universidade Federal de São Paulo (UNIFESP), São Paulo, 2017.