EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis

2017 ◽  
Vol 12 (4) ◽  
pp. 1526-1535 ◽  
Author(s):  
Martina Doubková ◽  
Jan Švancara ◽  
Michal Svoboda ◽  
Martina Šterclová ◽  
Vladimír Bartoš ◽  
...  
CHEST Journal ◽  
1997 ◽  
Vol 111 (1) ◽  
pp. 7-8 ◽  
Author(s):  
Steven H. Kirtland ◽  
Richard H. Winterbauer

2005 ◽  
Vol 59 (1) ◽  
pp. 77
Author(s):  
Sun Young Kyung ◽  
Cheul Hee Park ◽  
Young-Hee Lim ◽  
Chang Hyeok An ◽  
Sang Pyo Lee ◽  
...  

2020 ◽  
Vol 14 (11) ◽  
pp. 997-1007
Author(s):  
Sofia A Moll ◽  
Ivo A Wiertz ◽  
Adriane DM Vorselaars ◽  
Pieter Zanen ◽  
Henk JT Ruven ◽  
...  

Aim: Cancer antigen 15-3 (CA 15-3) is a baseline biomarker in idiopathic pulmonary fibrosis (IPF), but its value during follow-up is unknown. Materials and methods: Associations between serum CA 15-3 and pulmonary function tests during 1-year follow-up were evaluated by a mixed model in 132 IPF treated with pirfenidone or nintedanib. Results: Increased baseline (median: 56 kU/l) and follow-up CA 15-3 levels were inversely associated with forced vital capacity and diffusing capacity of the lung for carbon monoxide (estimates respectively: -5.21 and -4.69; p < 0.001). Baseline and 6-month CA 15-3 above 58.5 (hazard ratio: 1.67; p = 0.031) and 50.5 kU/l (hazard ratio: 2.99; p < 0.001), respectively, showed impaired survival compared with lower levels. Conclusion: CA 15-3 is associated with pulmonary function test during follow-up in IPF on antifibrotic treatment. Higher (follow-up) values are related with poor survival. Therefore, CA 15-3 is a promising follow-up biomarker in IPF.


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Byoung Soo Kwon ◽  
Jooae Choe ◽  
Kyung Hyun Do ◽  
Hee Sang Hwang ◽  
Eun Jin Chae ◽  
...  

Abstract Background A new clinical guideline for idiopathic pulmonary fibrosis (IPF) uses high-resolution computed tomography (HRCT) patterns for diagnostic purposes. However, it is unknown how they relate to the IPF clinical course. We aimed to investigate whether HRCT patterns could be used to predict lung function changes and survival in patients with IPF. Methods Clinical data were retrospectively reviewed in 337 patients with IPF (all biopsy-proven cases). HRCT patterns were classified according to the 2018 IPF diagnostic criteria. Results The median follow-up was 46.9 months. The mean age was 62.5 years, and 74.2% were men. Among the HRCT patterns, usual interstitial pneumonia (UIP), probable UIP, indeterminate for UIP, and an alternative diagnosis were identified in 163 (48.4%), 110 (32.6%), 33 (9.8%), and 31 (9.2%) patients, respectively. The indeterminate for UIP group showed higher lung function and exercise capacity and better prognosis than the other groups. They also had a lesser decline in lung function than the other groups during follow-up. In the multivariate Cox analysis, which was adjusted by age, smoking status, lung function, exercise capacity, and use of antifibrotic agents, indeterminate for UIP pattern was found to be an independent prognostic factor (hazard ratio 0.559, 95% confidence interval 0.335–0.933, P = 0.026). However, the probable UIP group had similar lung function changes and prognosis when compared the UIP group. Conclusions Our results suggest that indeterminate for UIP pattern on HRCT may predict a more favorable clinical course in patients with IPF, supporting the validity of the new IPF diagnostic guidelines.


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