scholarly journals P16‐72: Effects of nintedanib on forced vital capacity categorical changes and disease progression in Japanese patients with progressive fibrosing interstitial lung diseases: subanalysis of inbuild study

Respirology ◽  
2021 ◽  
Vol 26 (S3) ◽  
pp. 479-479
Keyword(s):  
Disease Progression ◽  
Forced Vital Capacity ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Japanese Patients ◽  
Interstitial Lung Diseases

scholarly journals Efficacy of early antifibrotic treatment for idiopathic pulmonary fibrosis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Keishi Sugino ◽  
Hirotaka Ono ◽  
Natsumi Watanabe ◽  
Masahiro Ando ◽  
Eiyasu Tsuboi ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Disease Progression ◽  
Vital Capacity ◽  
Early Stage ◽  
Japanese Patients ◽  
Oxygen Desaturation ◽  
Stage I ◽  
Relative Decline ◽  
Antifibrotic Drugs

Abstract Background Although antifibrotic drugs, including nintedanib and pirfenidone, slow the progression of idiopathic pulmonary fibrosis (IPF), there is little data about the timing of start of antifibrotic treatment in real-world clinical practice. The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF. Methods We compared survival and disease progression between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-min walk test (6MWT) and increased the gender–age–physiology (GAP) staging. We examined the efficacy of antifibrotic drugs in patients with early-stage IPF. Results The severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the relative decline in percentage predicted forced vital capacity (%FVC) over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients. Conclusion Antifibrotic drugs have a beneficial effect on the decline in %FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

The Gender-Age-Physiology (GAP) Index enhanced with KL-6 serum level for predicting disease progression in interstitial lung diseases (ILD)

2020 ◽  
Author(s):  
Francesco Bonella ◽  
Hegedüs Balasz ◽  
Eva Gottstein ◽  
Dirk Theegarten ◽  
Ulrich Costabel ◽  
...  
Keyword(s):  
Serum Level ◽  
Disease Progression ◽  
Lung Diseases ◽  
Interstitial Lung Diseases

scholarly journals Efficacy of Early Antifibrotic Treatment for Idiopathic Pulmonary Fibrosis

2021 ◽  
Author(s):  
Keishi Sugino ◽  
Hirotaka Ono ◽  
Natsumi Watanabe ◽  
Shigeru Shimizu ◽  
Masahiro Ando ◽  
...  
Keyword(s):  
Disease Progression ◽  
Vital Capacity ◽  
Early Stage ◽  
Japanese Patients ◽  
Oxygen Desaturation ◽  
Stage I ◽  
Walk Test ◽  
Relative Decline ◽  
6 Minute Walk Test ◽  
Minute Walk

Abstract The present study aimed to clarify the efficacy of nintedanib and pirfenidone in patients with early-stage IPF. We compared survival, disease progression, and efficacy of antifibrotics between patients with IPF with Japanese Respiratory Society (JRS) disease severity system stage I with and without oxygen desaturation on the 6-minute walk test (6MWT) and increased the gender–age–physiology (GAP) staging. The severity of stage I IPF (n = 179) according to the JRS criteria consisted of the following GAP staging criteria: stage I, 111 cases; stage II, 58 cases; stage III, 10 cases. The duration from the initial visit to disease progression and survival time was significantly shorter in JRS stage I patients with oxygen desaturation on the 6MWT or with increased GAP staging (unfavorable group) compared with patients without those factors. In the unfavorable group, the forced vital capacity (FVC) decline and relative decline in percentage predicted FVC over 6 months was significantly lower in patients undergoing antifibrotic treatment compared with non-treated patients. Antifibrotics have a beneficial effect on the decline in FVC in Japanese patients with early-stage IPF who have oxygen desaturation on the 6MWT or increased GAP staging.

scholarly journals Exclusion Spirometry: A Good Idea

2004 ◽  
Vol 11 (3) ◽  
pp. 185-186
Author(s):  
Nick R. Anthonise
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Primary Care ◽  
Pulmonary Disease ◽  
Forced Vital Capacity ◽  
Primary Care Physicians ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease

In this issue of theCanadian Respiratory Journal, Almirall and Bégin (pages 195 to 196) make a suggestion aimed at increasing the use of spirometry by primary care physicians, as well as family and general practitioners. The idea is that spirometry should be performed not necessarily to make specific diagnoses, but to rule in or out the possibility of a number of lung diseases, most notably, chronic obstructive pulmonary disease (COPD). If the patient demonstrates normal forced vital capacity and forced expiratory volume in 1 s, then he or she does not have COPD; if the results are not normal, then COPD is a possibility that can be further investigated.

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 Role of imaging in progressive-fibrosing interstitial lung diseases

2018 ◽  
Vol 27 (150) ◽  
pp. 180073 ◽  
Author(s):  
Simon L.F. Walsh ◽  
Anand Devaraj ◽  
Juan Ignacio Enghelmayer ◽  
Kazuma Kishi ◽  
Rafael S. Silva ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Disease Progression ◽  
Lung Diseases ◽  
Quantitative Methods ◽  
Visual Analysis ◽  
Imaging Techniques ◽  
Interstitial Lung Diseases ◽  
Diagnostic Process ◽  
Left Heart Failure ◽  
Specificity And Sensitivity

Imaging techniques are an essential component of the diagnostic process for interstitial lung diseases (ILDs). Chest radiography is frequently the initial indicator of an ILD, and comparison of radiographs taken at different time points can show the rate of disease progression. However, radiography provides only limited specificity and sensitivity and is primarily used to rule out other diseases, such as left heart failure. High-resolution computed tomography (HRCT) is a more sensitive method and is considered central in the diagnosis of ILDs. Abnormalities observed on HRCT can help identify specific ILDs. HRCT also can be used to evaluate the patient's prognosis, while disease progression can be assessed through serial imaging. Other imaging techniques such as positron emission tomography-computed tomography and magnetic resonance imaging have been investigated, but they are not commonly used to assess patients with ILDs. Disease severity may potentially be estimated using quantitative methods, as well as visual analysis of images. For example, comprehensive assessment of disease staging and progression in patients with ILDs requires visual analysis of pulmonary features that can be performed in parallel with quantitative analysis of the extent of fibrosis. New approaches to image analysis, including the application of machine learning, are being developed.

scholarly journals OP0272 68GA-FAPI-04 PET/CT STUDY EXTENSION FOR THE ASSESSMENT OF FIBROBLAST ACTIVATION AND RISK EVALUATION IN SYSTEMIC SCLEROSIS-RELATED INTERSTITIAL LUNG DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 165.1-166
Author(s):  
C. Bergmann ◽  
J. H. W. Distler ◽  
C. Treutlein ◽  
K. Tascilar ◽  
A. T. Mueller ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Disease Progression ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Control Group ◽  
Research Support ◽  
Fibroblast Activation ◽  
Pet Ct

Background:Interstitial lung disease (ILD) is the most common cause of death in systemic sclerosis (SSc). To date, the progression of SSc-ILD is judged by the accrual of lung damage on computed tomography (CT) and functional decline (forced vital capacity). However, this approach does not directly assess the activity of tissue remodeling. Moreover, prediction of the course of ILD in individual SSc patients remains challenging. Fibroblast Activation Protein (FAP) is a specific, ex vivo validated marker for activated fibroblasts.Objectives:The aims of this study were: 1. To assess differences in the uptake of 68GA-FAPI 04 in SSc-ILD patients compared to controls, to analyze 2. whether 68GA-FAPI 04 uptake at baseline correlates with other risk factors of disease progression and 3. Whether 68GA-FAPI 04 uptake is associated with the course of SSc-ILD.Methods:Between September 2018 and April 2020, 21 patients with SSc-ILD confirmed by HRCT and onset of SSc-ILD within ≤ 5 years or signs of progressive ILD and 21 controls without ILD were consecutively enrolled. All participants underwent 68Ga-FAPI-04 PET/CT imaging and standard-of-care procedures including HRCT and lung function testing (PFT) at baseline. Patients with SSc-ILD patients were followed-up for 6 months with HRCT and PFT. Follow-up 68Ga-FAPI-04 PET/CT scans were obtained in a subset of patients treated with nintedanib. We compared baseline 68Ga-FAPI-04 PET/CT uptake to standard diagnostic tools and currently used predictors of ILD progression. The association of 68Ga-FAPI-04 uptake with changes in FVC was analyzed using mixed-effects models.Results:68Ga-FAPI-04 accumulated in fibrotic areas of the lungs in SSc-ILD compared to controls with a median (q1-q3 interval) wlSUVmean of 0.8 (0.6 to 2.1) in the SSc-ILD group and 0.5 (0.4 to 0.5) in the control group (p<0.0001 with Mann-Whitney test) and a median whole lung maximal standardized uptake value (wlSUVmax) of 4.4 (3.05 to 5.2) in the SSc-ILD group compared to 0.7 (0.65 to 0.7) in the control group (p<0.0001). wlFAPI-MAV and wlTL-FAPI were not measurable in control subjects, as no 68Ga-FAPI-04 uptake above background level was observed. In the SSc-ILD group the median wlFAPI-MAV was 254cm3 (163.4 to 442.3) and the median wlTL-FAPI was 183.6 cm3 (98.04 to 960.7). 68Ga-FAPI-04 uptake was higher in patients with extensive disease, with previous ILD progression or high EUSTAR activity scores. Increased 68Ga-FAPI-04 uptake at baseline was associated with progression of ILD independently of extent of involvement on HRCT scan and the forced vital capacity at baseline. In consecutive 68Ga-FAPI-04-PET/CTs, changes in 68Ga-FAPI-04 uptake was concordant with the observed response to the fibroblast-targeting antifibrotic agent nintedanib.Conclusion:Our study presents first in human evidence that 68Ga-FAPI-04-fibroblast uptake correlates with fibrotic activity and disease progression in the lungs of SSc-ILD patients and that 68Ga-FAPI-04-PET/CT may be of potential to improve risk assessment of SSc-ILD.Figure 1.A and B:68Ga-FAPI-04 PET/CT scan from a patient with SSc-ILD with selective 68Ga-FAPI-04 uptake in fibrotic areas of the left- and right lower lung lobes (red arrows), but not in non-fibrotic areas such as the middle lobe (green arrow). B Corresponding CT component.Acknowledgements:We gratefully acknowledge Prof. Uwe Haberkorn (University Hospital Heidelberg and DKFZ, Heidelberg, Germany) and iTheranostics Inc. (Dulles, VA, USA) for providing the precursor FAPI-04.Disclosure of Interests:Christina Bergmann: None declared, Jörg H.W. Distler Speakers bureau: Actelion, Anamar, ARXX, Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, JB Therapeutics, and UCB, Grant/research support from: Anamar, Active Biotech, Array Biopharma, ARXX, aTyr, BMS, Bayer Pharma, Boehringer Ingelheim, Celgene, Galapagos, GSK, Inventiva, Novartis, Sanofi-Aventis, RedX, UCB, Christoph Treutlein: None declared, Koray Tascilar Speakers bureau: Gilead sciences GmbH, Pfizer Turkey, UCB Turkey, Anna-Theresa Mueller: None declared, Armin Atzinger: None declared, Alexandru-Emil Matei: None declared, Johannes Knitza: None declared, Andrea-Hermina Györfi: None declared, Anja Lueck: None declared, Clara Dees: None declared, Alina Soare: None declared, Andreas Ramming: None declared, Verena Schönau: None declared, Oliver Distler Speakers bureau: Arxx Therapeutics, Baecon Discovery, Blade Therapeutics,Bayer, Böhringer Ingelheim, Catenion,Competitive Drug Development International Ltd, Corbuspharma, CSL Behring, ChemomAb, Horizon Pharmaceuticals, Ergonex, Galaapagos NV, Glenmark Pharmaceuticals,GSK, Inventiva, Italfarmaco, IQvia, Kymera, Lilly, Medac, Medscape, MSD, Novartis, Pfizer, Roche, Sanofi, Taget Bio Sciencec, UCB, Grant/research support from: Bayer,Böhringer Ingelheim, Mitsubishi Tanabe Pharma, Olaf Prante: None declared, Philipp Ritt: None declared, Theresa Ida Goetz: None declared, Markus Koehner: None declared, Michael Cordes: None declared, Tobias Baeuerle: None declared, Torsten Kuwert Speakers bureau: Honoraria for occasional lectures by Siemens Healthineers, Grant/research support from: Research grant to the Clinic of Nuclear Medicine by this entity covering projects in the field of SPECT/CT, Georg Schett: None declared, Christian Schmidkonz: None declared

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