scholarly journals Pulmonary Vascular Volume is Associated With DLCO and Fibrotic Score in Idiopathic Pulmonary Fibrosis: An Observational Study

Author(s):  
Wen-Jui Wu ◽  
Wei-Ming Huang ◽  
Chia-Hao Liang ◽  
Chun-Ho Yun

Abstract Background: Idiopathic pulmonary fibrosis (IPF) is primarily a disease of old age. However, it is difficult to diagnose and has a complex disease course. High-resolution computed tomography (HRCT) and lung function test are crucial for its diagnosis and follow-up. However, the correlation of HRCT findings to lung function test results was not extensively investigated.Methods: This study retrospectively analysed the medical records and images of patients with IPF. Patients with evident emphysema and lung cancer were excluded. All included cases would be investigated through a multidiscipline discussion to confirm the diagnosis. The correlation of CT findings including fibrotic score, CT lung volume, pulmonary artery trunk (PA) diameter and pulmonary vascular volume (PVV) to the lung function test such as forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) was analysed.Results: A total of 32 patients were included. The fibrotic score and PVV were significantly correlated with DLCO (r = 0.59, p = 0.01; r = −0.43, p = 0.03, respectively) but not with FVC. The PVV was significantly correlated with fibrotic score (r = 0.59, p<0.01) and PA diameter (r = 0.47, p = 0.006).Conclusion: Our study shows the structural and functional correlation of IPF. The extent of lung fibrosis (fibrotic score) and PVV were associated with DLCO but not with FVC, especially for patients with IPF without significant FVC deficiency. The PA diameter, which reflects the pulmonary artery pressure, was associated with PVV.

2013 ◽  
Vol 107 (11) ◽  
pp. 1781-1788 ◽  
Author(s):  
Aline Bodlet ◽  
Gisèle Maury ◽  
Jacques Jamart ◽  
Caroline Dahlqvist

Respirology ◽  
2018 ◽  
Vol 23 (8) ◽  
pp. 764-770 ◽  
Author(s):  
Sylvia Nyilas ◽  
Theresa Schreder ◽  
Florian Singer ◽  
Alexander Poellinger ◽  
Thomas Kurt Geiser ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiyue Zhang ◽  
Wei Li ◽  
Chunyan Li ◽  
Jie Zhang ◽  
Zhenzhong Su

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with unclear pathogenesis. IPF is considered as a risk factor for lung cancer. Compared to other lung cancers, small-cell lung cancer (SCLC) has a lower incidence, but has a more aggressive course. Patients with IPF and SCLC have a lower survival rate, more difficult treatment, and poorer prognosis. Case presentation Case 1 was of a 66-year-old man with IPF for 5 years, who was admitted to our hospital for dyspnea. Case 2 was of a 68-year-old woman, who presented with chest pains, cough, and dyspnea. Both patients had extremely poor lung function. High-resolution computed tomography and pathology revealed that both patients had IPF and SCLC. Chemotherapy comprising nedaplatin (80 mg/m2) and etoposide (100 mg for 5 days) was initiated for both patients. Antifibrotic agents were continued during the chemotherapeutic regimen. Both patients showed improvement in their condition after treatment. Conclusion The favorable outcomes in these 2 cases suggests that chemotherapy is worth considering in the management of patients having SCLC and IPF with poor lung function.


Author(s):  
Gaetano Rea ◽  
Marina De Martino ◽  
Annalisa Capaccio ◽  
Pasquale Dolce ◽  
Tullio Valente ◽  
...  

Abstract Background Volumetric high-resolution computed tomography (HRCT) of the chest has recently replaced incremental CT in the diagnostic workup of idiopathic pulmonary fibrosis (IPF). Concomitantly, visual and quantitative scores have been proposed for disease extent assessment to ameliorate disease management. Purpose To compare the performance of density histograms (mean lung attenuation, skewness, and kurtosis) and visual scores, along with lung function correlations, in IPF patients submitted to incremental or volumetric thorax HRCT. Material and methods Clinical data and CT scans of 89 newly diagnosed and therapy-naive IPF patients were retrospectively evaluated. Results Forty-six incremental and 43 volumetric CT scans were reviewed. No differences of density histograms and visual scores estimates were found by comparing two HRCT techniques, with an optimal inter-operator agreement (concordance correlation coefficient >0.90 in all instances). Single-breath diffusing lung capacity for carbon monoxide (DLCOsb) was inversely related with the Best score (r = −00.416; p = 0.014), the Kazerooni fibrosis extent (r = −0.481; p = 0.004) and the mean lung attenuation (r = −0.382; p = 0.026), while a positive correlation was observed with skewness (r = 0.583; p = 0.001) and kurtosis (r = 0.543; p = 0.001) in the incremental HRCT sub-group. Similarly, in the volumetric CT sub-cohort, DLCOsb was significantly associated with skewness (r = 0.581; p = 0.007) and kurtosis (r = 0.549; p = 0.018). Correlations with visual scores were not confirmed. Forced vital capacity significantly related to all density indices independently on HRCT technique. Conclusions Density histograms and visual scores similarly perform in incremental and volumetric HRCT. Density quantification displays an optimal reproducibility and proves to be superior to visual scoring as more strongly correlated with lung function.


Pneumologie ◽  
2018 ◽  
Vol 72 (S 01) ◽  
pp. S47-S48
Author(s):  
U Costabel ◽  
C Albera ◽  
KU Kirchgaessler ◽  
F Gilberg ◽  
U Petzinger ◽  
...  

Author(s):  
Jung Keun Choi ◽  
Mi A Son ◽  
Hyun Kyung Kim ◽  
Domyung Paek ◽  
Byung Soon Choi

Respiration ◽  
2021 ◽  
pp. 1-34
Author(s):  
Jürgen Behr ◽  
Andreas Günther ◽  
Francesco Bonella ◽  
Julien Dinkel ◽  
Ludger Fink ◽  
...  

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.


2021 ◽  
Vol 17 (1) ◽  
Author(s):  
Elodie Roels ◽  
Aline Fastrès ◽  
Anne-Christine Merveille ◽  
Géraldine Bolen ◽  
Erik Teske ◽  
...  

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.


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