scholarly journals Does Technetium-99m Diethylenetriaminepentaacetate Clearance Predict the Clinical Course of Idiopathic Pulmonary Fibrosis?

2004 ◽  
Vol 11 (7) ◽  
pp. 477-479 ◽  
Author(s):  
Marco Mura ◽  
Gelorma Belmonte ◽  
Stefano Fanti ◽  
Angela Maria Pacilli ◽  
Luca Fasano ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Clinical Course ◽  
Pulmonary Function Tests ◽  
Functional Tests ◽  
High Resolution Computed Tomography ◽  
Technetium 99M ◽  
Potential Indicator

Clearance of inhaled technetium-99m diethylenetriaminepentaacetate (99mTc-DTPA) is a potential indicator of disease activity and progression in idiopathic pulmonary fibrosis (IPF). The objective of the present study was to evaluate the prognostic value of99mTc-DTPA scans in IPF. A total of 22 patients (18 males), aged 33 to 80 years with IPF were followed for six to 20 months (mean 13 months). At diagnosis, high resolution computed tomography (HRCT) scans showed a honeycomb pattern with bibasilar reticular opacities in all cases. At T0 (diagnosis) and T1 (follow-up), each patient had pulmonary function tests (forced vital capacity, diffusing capacity of the lung for carbon monoxide and partial arterial O2pressure), extension of fibrosis evaluated by HRCT visual score and99mTc-DTPA lung clearance. Results at T0 and T1 were compared, taking into account the whole population and patients with relatively fast and slow99mTc-DTPA wash-out.99mTc-DTPA clearance did not show any significant correlation with functional tests or HRCT score. These findings indicate that clearance of inhaled99mTc-DTPA is not of value in following the progress of IPF.

scholarly journals Serum biomarker CA 15-3 as predictor of response to antifibrotic treatment and survival in idiopathic pulmonary fibrosis

2020 ◽  
Vol 14 (11) ◽  
pp. 997-1007
Author(s):  
Sofia A Moll ◽  
Ivo A Wiertz ◽  
Adriane DM Vorselaars ◽  
Pieter Zanen ◽  
Henk JT Ruven ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Function ◽  
Vital Capacity ◽  
Mixed Model ◽  
Pulmonary Function Tests ◽  
Hazard Ratio ◽  
Poor Survival ◽  
Function Test

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.

scholarly journals Histologic features suggesting connective tissue disease in idiopathic pulmonary fibrosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Ho Cheol Kim ◽  
Joon Seon Song ◽  
Sojung Park ◽  
Hee-Young Yoon ◽  
So Yun Lim ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Clinical Course ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Cox Analysis

AbstractSome patients with idiopathic pulmonary fibrosis (IPF) have histopathologic features suggesting connective tissue disease (CTD); however, their clinical course and prognosis remain unclear. Thus, we aimed to investigate the clinical course and prognosis of these patients with histologic autoimmune features. Among 114 patients with biopsy-proven IPF, the histologic features were semi-quantitatively graded, and CTD scores (range: 0–9) were calculated as the sum of each score of plasma cell infiltration, lymphoid aggregates, and germinal centres. Patients with high CTD scores (≥ 4) were classified into the interstitial pneumonia with histologic autoimmune features (IP-hAF) group. The mean age of the patients was 60.0 years; 74.6% were men, 69.3% were ever-smokers, and 35.1% had IP-hAF. During follow-up, the IP-hAF group showed slower decline in lung function, and better prognosis (median survival, 48.7 vs. 40.4 months; p = 0.015) than the no-IP-hAF group. On multivariate Cox analysis, IP-hAF was an independent prognostic factor (hazard ratio, 0.522; p = 0.016), along with the lower diffusing capacity for carbon monoxide, higher scores of reticulation and honeycombing, and usual interstitial pneumonia pattern on high-resolution computed tomography. Patients with IPF having histologic autoimmune features show distinct clinical characteristics and better outcome than those without histologic autoimmune features.

scholarly journals Computed tomography patterns predict clinical course of idiopathic pulmonary fibrosis

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Byoung Soo Kwon ◽  
Jooae Choe ◽  
Kyung Hyun Do ◽  
Hee Sang Hwang ◽  
Eun Jin Chae ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Exercise Capacity ◽  
Clinical Course ◽  
Usual Interstitial Pneumonia ◽  
The Other ◽  
Hrct Patterns

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.

scholarly journals Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Makoto Shioya ◽  
Mitsuo Otsuka ◽  
Gen Yamada ◽  
Yasuaki Umeda ◽  
Kimiyuki Ikeda ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Total Lung Capacity ◽  
Unknown Etiology ◽  
Advanced Stage ◽  
High Resolution Computed Tomography ◽  
Pleuroparenchymal Fibroelastosis ◽  
Radiological Criteria ◽  
Lung Capacity

Objective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods. We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results. Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions. Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE.

scholarly journals Adherence to guidelines in idiopathic pulmonary fibrosis: a follow-up national survey

2015 ◽  
Vol 1 (2) ◽  
pp. 00032-2015 ◽  
Author(s):  
Vincent Cottin ◽  
Emmanuel Bergot ◽  
Arnaud Bourdin ◽  
Jacques Cadranel ◽  
Philippe Camus ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Substantial Improvement ◽  
Diffusing Capacity ◽  
Adherence To Guidelines ◽  
Therapeutic Practice ◽  
Practical Guidelines ◽  
E Mail

A new survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF) and explored changes since a previous survey in 2011–2012.From May 16 to August 30, 2014, 524 pulmonologists were contacted. Those following at least one patient with IPF were invited to complete a questionnaire administered by telephone or e-mail.166 (31.7%) pulmonologists, 161 (97%) of whom had participated to the first survey, completed the questionnaire. Of those, 46% and 52%, respectively, discussed the cases with radiologists and pathologists. Out of 144 pulmonologists practicing outside of expert centres, 80% indicated referring patients to those centres. The 2013 French practical guidelines for IPF were known by 92% of pulmonologists involved in IPF, 96% of whom considered them appropriate for practice. The multidisciplinary discussion form for IPF diagnosis was known by 74% and considered appropriate by 94%. Diagnosis and management resulted from multidisciplinary discussion in 50% of the cases. About 58% of patients were diagnosed with “mild to moderate IPF” as defined by forced vital capacity ≥50% of the predicted value and diffusing capacity for carbon monoxide ≥35% of predicted. At the time of the survey, 31% of physicians were using pirfenidone to treat patients with “mild-to-moderately severe IPF” and 30% generally prescribed no treatment.Substantial improvement has occurred since the 2011–2012 survey with regard to knowledge of guidelines and proper management of IPF. Early diagnosis still needs to be improved through the network of expert centres.

scholarly journals Automatic quantitative computed tomography measurement of longitudinal lung volume loss in interstitial lung diseases

2022 ◽  
Author(s):  
Salim A. Si-Mohamed ◽  
Mouhamad Nasser ◽  
Marion Colevray ◽  
Olivier Nempont ◽  
Pierre-Jean Lartaud ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Volume Loss ◽  
Lung Capacity ◽  
Lung Ct ◽  
Ct Volume

Abstract Objectives To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann–Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated. Results In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p < 0.0001). In 408 patients (3332 CT), median annual CTvol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p < 0.0001) over 5.03 years. In 73 IPF patients, a relative annual CTvol loss of 7.9% was associated with major adverse events (log-rank, p < 0.0001) in univariate analysis (p < 0.001). Conclusions Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD. Key Points • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p < 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p < 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p < 0.0001).

scholarly journals Importance of serial changes in biomarkers in idiopathic pulmonary fibrosis

2017 ◽  
Vol 3 (3) ◽  
pp. 00019-2016 ◽  
Author(s):  
Akihiko Sokai ◽  
Kiminobu Tanizawa ◽  
Tomohiro Handa ◽  
Kumiko Kanatani ◽  
Takeshi Kubo ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Surfactant Protein ◽  
Surfactant Protein D ◽  
Prognostic Information ◽  
The Mean ◽  
Serial Measurements ◽  
Overall Mortality

The clinical significance of serial changes in serum biomarkers in patients with idiopathic pulmonary fibrosis (IPF) remains to be established. This retrospective study was conducted to clarify the associations of serial changes in serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) with changes in physiological indices and overall mortality in IPF.The study subjects were 75 patients with IPF. The 6 month change in serum KL-6 was significantly correlated with changes in the percentage of the predicted forced vital capacity (FVC % pred) and the percentage of the predicted diffusing capacity of the lung for carbon monoxide (% DLCO), while the 6 month change in serum SP-D was correlated only with % DLCO. During the mean follow-up period of 647 days, 22 (29.3%) patients died. An increase in serum KL-6 over a 6 month period was a significant predictor of mortality even after adjustment for %FVC, % DLCO and serum KL-6 at the baseline (hazard ratio 1.10 per 100 U·mL−1, 95% CI 1.01–1.18, p=0.03), whereas the 6 month increase in serum SP-D was not significant.Serial measurements of serum KL-6 may provide additional prognostic information compared to that provided by physiological parameters in patients with IPF.

scholarly journals The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis

2019 ◽  
Vol 16 ◽  
pp. 147997311986794 ◽  
Author(s):  
Hiroshi Ishii ◽  
Yoshiaki Kinoshita ◽  
Hisako Kushima ◽  
Nobuhiko Nagata ◽  
Kentaro Watanabe
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Vital Capacity ◽  
Marked Decrease ◽  
Clinical Course ◽  
Walking Distance ◽  
Thoracic Cage ◽  
Pleuroparenchymal Fibroelastosis ◽  
Current Classification

The idiopathic form of pleuroparenchymal fibroelastosis (PPFE) is categorized as a rare idiopathic interstitial pneumonia in the current classification. The majority of PPFE cases are idiopathic, but many predisposing factors or comorbidities have been reported. Although histological PPFE is predominantly located in the upper lobes, which are less often affected by fibrosis in patients with idiopathic pulmonary fibrosis (IPF), the clinical course of PPFE is seemingly similar to that of IPF. However, upper lobe fibroelastosis has various clinical and physiological characteristics that differ from those of IPF, including a flattened thoracic cage and a marked decrease in the forced vital capacity (FVC) but with a preserved residual volume. Compared with IPF, the decrease in the walking distance is mild despite the markedly decreased FVC in PPFE, and chest radiograph more frequently shows the elevation of bilateral hilar opacities with or without tracheal deviation. The prognosis may be related to the development of fibrosing interstitial pneumonia in the lower lobes with elevated levels of serum Krebs von den Lungen-6; however, there is marked variation in the pathogenesis and clinical features in PPFE. A proposal of the diagnostic criteria for idiopathic PPFE with and without surgical lung biopsy, which has recently been published, may be useful.

scholarly journals Quantitative high-resolution computed tomography fibrosis score: performance characteristics in idiopathic pulmonary fibrosis

2018 ◽  
Vol 52 (3) ◽  
pp. 1801384 ◽  
Author(s):  
Stephen M. Humphries ◽  
Jeffrey J. Swigris ◽  
Kevin K. Brown ◽  
Matthew Strand ◽  
Qi Gong ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Fibrosis ◽  
Important Change ◽  
Performance Characteristics ◽  
High Resolution Computed Tomography ◽  
Clinically Important Change

We evaluated performance characteristics and estimated the minimal clinically important difference (MCID) of data-driven texture analysis (DTA), a high-resolution computed tomography (HRCT)-derived measurement of lung fibrosis, in subjects with idiopathic pulmonary fibrosis (IPF).The study population included 141 subjects with IPF from two interventional clinical trials who had both baseline and nominal 54- or 60-week follow-up HRCT. DTA scores were computed and compared with forced vital capacity (FVC), diffusing capacity of the lung for carbon monoxide, distance covered during a 6-min walk test and St George's Respiratory Questionnaire scores to assess the method's reliability, validity and responsiveness. Anchor- and distribution-based methods were used to estimate its MCID.DTA had acceptable reliability in subjects appearing stable according to anchor variables at follow-up. Correlations between the DTA score and other clinical measurements at baseline were moderate to weak and in the hypothesised directions. Acceptable responsiveness was demonstrated by moderate to weak correlations (in the directions hypothesised) between changes in the DTA score and changes in other parameters. Using FVC as an anchor, MCID was estimated to be 3.4%.Quantification of lung fibrosis extent on HRCT using DTA is reliable, valid and responsive, and an increase of ∼3.4% represents a clinically important change.

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