Idiopathic Pulmonary Fibrosis
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.
KeywordsIdiopathic pulmonary fibrosis (IPF) Usual Interstitial Pneumonitis (UIP) Patchy fibrosis Fibroblastic foci Honeycomb changes Transbronchial cryobiopsy
- 6.Raghu G, Flaherty KR, Lederer DJ, Lynch DA, Colby TV, et al. Use of molecular classifier to identify usual interstitial pneumonia in conventional transbronchial lung biopsy samples: a prospective validation study. Lancet Respir Med. 2019. In press.Google Scholar