Imaging in idiopathic pulmonary fibrosis: diagnosis and mimics

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Citações na Scopus
27
Tipo de produção
article
Data de publicação
2019
Título da Revista
ISSN da Revista
Título do Volume
Editora
HOSPITAL CLINICAS, UNIV SAO PAULO
Autores
HOCHHEGGER, Bruno
MARCHIORI, Edson
ZANON, Matheus
RUBIN, Adalberto Sperb
FRAGOMENI, Renata
ALTMAYER, Stephan
Citação
CLINICS, v.74, article ID e225, 7p, 2019
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Idiopathic pulmonary fibrosis is a chronic disease of unknown etiology that usually has a progressive course and is commonly associated with a poor prognosis. The main symptoms of idiopathic pulmonary fibrosis, including progressive dyspnea and dry cough, are often nonspecific. Chest high-resolution computed tomography is the primary modality used in the initial assessment of patients with suspected idiopathic pulmonary fibrosis and may have considerable influence on subsequent management decisions. The main role of computed tomography is to distinguish chronic fibrosing lung diseases with a usual interstitial pneumonia pattern from those presenting with a non-usual interstitial pneumonia pattern, suggesting an alternative diagnosis when possible. A usual interstitial pneumonia pattern on chest tomography is characterized by the presence subpleural and basal predominance, reticular abnormality honeycombing with or without traction bronchiectasis, and the absence of features suggestive of an alternative diagnosis. Idiopathic pulmonary fibrosis can be diagnosed according to clinical and radiological criteria in approximately 66.6% of cases. Confirmation of an idiopathic pulmonary fibrosis diagnosis is challenging, requiring the exclusion of pulmonary fibroses with known causes, such as asbestosis, connective tissue diseases, drug exposure, chronic hypersensitivity pneumonitis, and other forms of idiopathic interstitial pneumonitis. The histopathological hallmark of usual interstitial pneumonia is a heterogeneous appearance, characterized by areas of fibrosis with scarring and honeycombing alternating with areas of less affected or normal parenchyma. The aim of this article was to review the clinical, radiological, and pathological features of idiopathic pulmonary fibrosis and of diseases that might mimic idiopathic pulmonary fibrosis presentation.
Palavras-chave
Differential Diagnosis, Idiopathic Pulmonary Fibrosis, Radiology, Pathology, Interstitial Lung Diseases
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