Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a peculiar (typical) HRCT pattern, but biopsy can demonstrate usual interstitial pneumonia in patients with atypical patterns. It is unknown how progression pattern varies among different radiographic presentations of IPF. We sought to investigate the longitudinal radiographic evolution and survival of typical and non-typical patterns. Materials and Methods: One-hundred-twenty-three patients diagnosed with IPF in 2 tertiary referral hospitals were included in the study. Longitudinal evolution of non-typical patterns was considered. The HRCT visual fibrosis score was used as a reliable evaluation tool of disease progression. HRCTs were scored by 2 senior chest radiologists with ILD expertise. The primary endpoint was the evolution of the presentation pattern to probable or typical. The secondary endpoint was lung transplant (LTx)-free survival from the time of diagnosis. Results: Average interval between HRCTs was 16±5 months; average follow-up after the 2nd HRCT was 17±11 months. Four out of 45 (8.9%) patients with probable pattern "evolved" to a typical pattern of IPF, while 5 out of 31 (16.1%) with indeterminate/alternative pattern "evolved" to probable pattern. An average HRCT fibrosis score increase of 9±11% was observed with typical (n=49), 6±5% with probable (n=43) and 7±8% (n=31) with indeterminate/alternative presentation pattern. LTx-free survival and lung function declines did not show any difference related to presentation HRCT patterns. Conclusions: The evolution of a non-typical UIP pattern to a typical one is infrequent. All presentation HRCT patterns of IPF evolve in similar way and are associated with comparable survival time.[/sc].
Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a peculiar (typical) HRCT pattern, but biopsy can demonstrate usual interstitial pneumonia in patients with atypical patterns. It is unknown how progression pattern varies among different radiographic presentations of IPF. We sought to investigate the longitudinal radiographic evolution and survival of typical and non-typical patterns. Materials and Methods: One-hundred-twenty-three patients diagnosed with IPF in 2 tertiary referral hospitals were included in the study. Longitudinal evolution of non-typical patterns was considered. The HRCT visual fibrosis score was used as a reliable evaluation tool of disease progression. HRCTs were scored by 2 senior chest radiologists with ILD expertise. The primary endpoint was the evolution of the presentation pattern to probable or typical. The secondary endpoint was lung transplant (LTx)-free survival from the time of diagnosis. Results: Average interval between HRCTs was 16±5 months; average follow-up after the 2nd HRCT was 17±11 months. Four out of 45 (8.9%) patients with probable pattern "evolved" to a typical pattern of IPF, while 5 out of 31 (16.1%) with indeterminate/alternative pattern "evolved" to probable pattern. An average HRCT fibrosis score increase of 9±11% was observed with typical (n=49), 6±5% with probable (n=43) and 7±8% (n=31) with indeterminate/alternative presentation pattern. LTx-free survival and lung function declines did not show any difference related to presentation HRCT patterns. Conclusions: The evolution of a non-typical UIP pattern to a typical one is infrequent. All presentation HRCT patterns of IPF evolve in similar way and are associated with comparable survival time.[/sc].
Authors: Hyo Jung Park; Sang Min Lee; Jin Woo Song; Sang Min Lee; Sang Young Oh; Namkug Kim; Joon Beom Seo Journal: AJR Am J Roentgenol Date: 2016-08-17 Impact factor: 3.959
Authors: Margaret L Salisbury; David A Lynch; Edwin J R van Beek; Ella A Kazerooni; Junfeng Guo; Meng Xia; Susan Murray; Kevin J Anstrom; Eric Yow; Fernando J Martinez; Eric A Hoffman; Kevin R Flaherty Journal: Am J Respir Crit Care Med Date: 2017-04-01 Impact factor: 21.405
Authors: Kevin R Flaherty; Jeanette A Mumford; Susan Murray; Ella A Kazerooni; Barry H Gross; Thomas V Colby; William D Travis; Andrew Flint; Galen B Toews; Joseph P Lynch; Fernando J Martinez Journal: Am J Respir Crit Care Med Date: 2003-05-28 Impact factor: 21.405
Authors: Ganesh Raghu; Martine Remy-Jardin; Jeffrey L Myers; Luca Richeldi; Christopher J Ryerson; David J Lederer; Juergen Behr; Vincent Cottin; Sonye K Danoff; Ferran Morell; Kevin R Flaherty; Athol Wells; Fernando J Martinez; Arata Azuma; Thomas J Bice; Demosthenes Bouros; Kevin K Brown; Harold R Collard; Abhijit Duggal; Liam Galvin; Yoshikazu Inoue; R Gisli Jenkins; Takeshi Johkoh; Ella A Kazerooni; Masanori Kitaichi; Shandra L Knight; George Mansour; Andrew G Nicholson; Sudhakar N J Pipavath; Ivette Buendía-Roldán; Moisés Selman; William D Travis; Simon Walsh; Kevin C Wilson Journal: Am J Respir Crit Care Med Date: 2018-09-01 Impact factor: 21.405