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Idiopathic Pulmonary Fibrosis

2018; Massachusetts Medical Society; Volume: 379; Issue: 8 Linguagem: Inglês

10.1056/nejmc1807508

1533-4406

Hedi Orbach, Maria Karlinskaya, Oren Fruchter,

Occupational and environmental lung diseases

Idiopathic pulmonary fibrosis is a severe disorder mainly affecting lungs. It occurs more frequently in smokers or former smokers with an average age of onset around 65. The first pathogenetic event leading to the complete derangement of lung parenchyma is the alveolar stem cell exhaustion due to intrinsic factors (genetic mutations or polymorphisms) and extrinsic insults (cigarette smoke, pollution). The histopathologic background is Usual Interstitial Pneumonitis. Transbronchial cryobiopsy allows harvesting of lung samples large enough to identify the characteristic of this morphologic pattern. The best strategy to maintain a high diagnostic yield is to biopsy at least two different segments.