Acute Exacerbations In Idiopathic Pulmonary Fibrosis Are Associated With Pulmonary Hypertension And Histological Acute Lung Injury In Explanted IPF Tissue

2010 ◽  
Author(s):  
Eoin P. Judge ◽  
Aurelie Fabre ◽  
Jim McCarthy ◽  
A. E. Wood ◽  
Jim J. Egan
Keyword(s):  
Pulmonary Hypertension ◽  
Acute Lung Injury ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Injury ◽  
Acute Exacerbations

scholarly journals Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis

2011 ◽  
Vol 40 (1) ◽  
pp. 93-100 ◽  
Author(s):  
Eoin P. Judge ◽  
Aurelie Fabre ◽  
Huzaifa I. Adamali ◽  
Jim J. Egan
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbations

scholarly journals Plasma biomarker profiles in acute exacerbation of idiopathic pulmonary fibrosis

2010 ◽  
Vol 299 (1) ◽  
pp. L3-L7 ◽  
Author(s):  
Harold R. Collard ◽  
Carolyn S. Calfee ◽  
Paul J. Wolters ◽  
Jin Woo Song ◽  
Sang-Bum Hong ◽  
...  
Keyword(s):  
Acute Lung Injury ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Injury ◽  
Acute Exacerbation ◽  
Cell Injury ◽  
Surfactant Protein D ◽  
Type Ii ◽  
Plasma Biomarker ◽  
Alveolar Epithelial

Little is known about the pathobiology of acute exacerbation of idiopathic pulmonary fibrosis (IPF), a condition that shares clinical and histopathological features with acute lung injury. Plasma biomarkers have been well studied in acute lung injury and have provided insight into the underlying disease mechanism. The objective of this study was to determine the plasma biomarker profile of acute exacerbation of IPF and compare this profile with that of stable IPF and acute lung injury. Plasma was collected from patients with stable IPF, acute exacerbation of IPF, and acute lung injury for measurement of biomarkers of cellular activity/injury (receptor for advanced glycation endproducts, surfactant protein D, KL-6, von Willebrand factor), systemic inflammation (IL-6), and coagulation/fibrinolysis (protein C, thrombomodulin, plasminogen activator inhibitor-1). Plasma from patients with acute exacerbation of IPF showed significant elevations in markers of type II alveolar epithelial cell injury and/or proliferation, endothelial cell injury, and coagulation. This profile differed from the biomarker profile in patients with acute lung injury. These findings support the hypothesis that type II alveolar epithelial cells are centrally involved in the pathobiology of acute exacerbation of IPF. Furthermore, they suggest that acute exacerbation of IPF has a distinct plasma biomarker profile from that of acute lung injury.

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis

2015 ◽  
Vol 1 (2) ◽  
pp. 79-86
Author(s):  
Juan Enghelmayer ◽  
Tulio Papucci ◽  
Alejandro Raimondi
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis

scholarly journals The prevalence of pulmonary hypertension assessed using the pulmonary vein‐to‐right pulmonary artery ratio and its association with survival in West Highland white terriers with canine idiopathic pulmonary fibrosis

2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Elodie Roels ◽  
Aline Fastrès ◽  
Anne-Christine Merveille ◽  
Géraldine Bolen ◽  
Erik Teske ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Artery ◽  
Pulmonary Vein ◽  
Survival Data ◽  
Study Objective ◽  
Time Motion ◽  
Co Morbidity ◽  
Right Pulmonary Artery

Abstract Background Pulmonary hypertension (PH) is a known co-morbidity in West Highland white terriers (WHWTs) affected with canine idiopathic pulmonary fibrosis (CIPF). The pulmonary vein-to-right pulmonary artery ratio (PV/PA) has recently been described for the detection of pre-capillary PH in dogs. The objective of the present study was to estimate the prevalence of PH at diagnostic, in WHWTs affected with CIPF, by using PV/PA, in comparison with a group of healthy breed-matched controls (CTRLs). Additional study objective was to explore whether the presence of PH at initial diagnosis of CIPF impacted survival time in dogs treated with sildenafil. Results Twenty-five client-owned WHWTs presented with CIPF and 19 CTRLs were included in the study. PV/PA in either two-dimensional mode (2D) or time-motion mode or both were measured from cineloops in each dog. Dogs were classified according to PV/PA value into non/mild PH (PV/PA measured in 2D ≥ 0.7) or moderate/severe PH (PV/PA < 0.7). Survival data of WHWTs affected with CIPF were extracted from medical record to assess association between presence of PH at diagnosis and outcome. 60 % overall prevalence for moderate/severe PH was estimated in this cohort of WHWTs presented with CIPF vs. 5 % in CTRLS (P = 0.0002). The presence of moderate/severe PH at initial presentation was not associated with survival. Conclusions Results of the present study confirm a high prevalence of PH at diagnosis in WHWTs affected with CIPF and highlight the utility of PV/PA as a non-invasive surrogate for assessment of PH in this population.

scholarly journals Initial therapeutic dose of corticosteroid for an acute exacerbation of IPF is associated with subsequent early recurrence of another exacerbation

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Ryo Yamazaki ◽  
Osamu Nishiyama ◽  
Sho Saeki ◽  
Hiroyuki Sano ◽  
Takashi Iwanaga ◽  
...  
Keyword(s):  
Risk Factors ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Therapeutic Dose ◽  
Early Recurrence ◽  
Time To Recurrence ◽  
Acute Exacerbations ◽  
Patient Groups ◽  
The Mean ◽  
Mean Time

AbstractSome patients with idiopathic pulmonary fibrosis (IPF) undergo recurrent acute exacerbations (AEs). This study aimed to elucidate the risk factors for recurrent AEs of IPF (AE-IPF). Consecutive patients with IPF admitted for their first AE-IPF between January 2008 and December 2018 were retrospectively recruited. Of 63 patients admitted for an AE-IPF and discharged alive, 9 (14.3%) developed a recurrence of AE within 1 year. The mean time to recurrence was 233 ± 103 days. Total doses (mg/month and mg/kg/month) of corticosteroids administered over day 1 to 30 after the AE were significantly higher in patients without recurrences of AE-IPF (5185 ± 2414 mg/month, 93.5 ± 44.0 mg/kg/month) than the doses in patients with recurrences (3133 ± 1990 mg/month, 57.2 ± 37.7 mg/kg/month) (p = 0.02 and p = 0.03, respectively). However, no differences were observed between the total doses of corticosteroids administered over days 31 to 60, 61 to 90, 91 to 120, and 151 to 180 after the AE. Furthermore, differences between the administration rates of immunosuppressive and antifibrotic treatments administered to the 2 patient groups were not significant. An increased total dose of corticosteroid administered over day 1 to 30 after an AE-IPF was associated with a decreased risk of subsequent recurrence of AE-IPF within 1 year after the first AE.

scholarly journals Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

Respiration ◽  
2011 ◽  
Vol 82 (3) ◽  
pp. 294-304 ◽  
Author(s):  
Georgia Pitsiou ◽  
Despina Papakosta ◽  
Demosthenes Bouros
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis
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