scholarly journals Autoimmune Progressive Fibrosing Interstitial Lung Disease: Predictors of Fast Decline

2021 ◽  
Vol 12 ◽  
Author(s):  
Alexandra Nagy ◽  
Tamas Nagy ◽  
Abigel Margit Kolonics-Farkas ◽  
Noemi Eszes ◽  
Krisztina Vincze ◽  
...  
Keyword(s):  
Lung Function ◽  
Functional Decline ◽  
Blood Gas Analysis ◽  
Interstitial Lung Diseases ◽  
Gas Analysis ◽  
Lung Function Decline ◽  
Post Exercise ◽  
Antifibrotic Therapy ◽  
Detailed Medical History ◽  
Fast Decline

A subset of interstitial lung diseases (ILDs) with autoimmune traits—including connective tissue disease-associated ILD (CTD-ILD) and interstitial pneumonia with autoimmune features (IPAF)—develops progressive fibrosing (PF)-ILD. The aim of our study was to evaluate the clinical characteristics and predictors of longitudinal lung function (LF) changes in autoimmune PF-ILD patients in a real-world setting. All ILD cases with confirmed or suspected autoimmunity discussed by a multidisciplinary team (MDT) between January 2017 and June 2019 (n = 511) were reviewed, including 63 CTD-ILD and 44 IPAF patients. Detailed medical history, LF test, diffusing capacity of the lung for carbon monoxide (DLCO), 6-min walk test (6MWT), blood gas analysis (BGA), and high-resolution computer tomography (HRCT) were performed. Longitudinal follow-up for functional parameters was at least 2 years. Women were overrepresented (70.1%), and the age of the IPAF group was significantly higher as compared to the CTD-ILD group (p < 0.001). Dyspnea, crackles, and weight loss were significantly more common in the IPAF group as compared to the CTD-ILD group (84.1% vs. 58.7%, p = 0.006; 72.7% vs. 49.2%, p = 0.017; 29.6% vs. 4.8%, p = 0.001). Forced vital capacity (FVC) yearly decline was more pronounced in IPAF (53.1 ± 0.3 vs. 16.7 ± 0.2 ml; p = 0.294), while the majority of patients (IPAF: 68% and CTD-ILD 82%) did not deteriorate. Factors influencing progression included malignancy as a comorbidity, anti-SS-A antibodies, and post-exercise pulse increase at 6MWT. Antifibrotic therapy was administered significantly more often in IPAF as compared to CTD-ILD patients (n = 13, 29.5% vs. n = 5, 7.9%; p = 0.007), and importantly, this treatment reduced lung function decline when compared to non-treated patients. Majority of patients improved or were stable regarding lung function, and autoimmune-associated PF-ILD was more common in patients having IPAF. Functional decline predictors were anti-SS-A antibodies and marked post-exercise pulse increase at 6MWT. Antifibrotic treatments reduced progression in progressive fibrosing CTD-ILD and IPAF, emphasizing the need for guidelines including optimal treatment start and combination therapies in this special patient group.

scholarly journals Chronic hypoxaemia and gender status modulate adiponectin plasmatic level and its multimer proportion in severe COPD patients: new endotypic presentation?

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Mélany Pierard ◽  
Alexandra Tassin ◽  
Antoine Legrand ◽  
Alexandre Legrand
Keyword(s):  
Lung Function ◽  
Pearson Correlation ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Lung Function Decline ◽  
Cvd Risk ◽  
Copd Patients ◽  
Gender Status ◽  
Plasmatic Level ◽  
And Gender

Abstract Background Disease progression in COPD patient is associated to lung function decline, leading to a higher risk of hypoxaemia and associated comorbidities, notably cardiovascular diseases (CVD). Adiponectin (Ad) is an adipokine with cardio-protective properties. In COPD patients, conflicting results were previously reported regarding Ad plasmatic (Adpl) level, probably because COPD is a heterogeneous disease with multifactorial influence. Among these factors, gender and hypoxaemia could interact in a variety of ways with Ad pathway. Therefore, we postulated that these components could influence Adpl level and its multimers in COPD patients and contribute to the appearance of a distinct endotype associated to an altered CVD risk. Methods One hundred COPD patients were recruited: 61 were men and 39 were women. Patients who were not severely hypoxemic were allocated to non-hypoxemic group which included 46 patients: 27 men and 19 women. Hypoxemic group included 54 patients: 34 men and 20 women. For all patients, Adpl level and proportion of its different forms were measured. Differences between groups were evaluated by Rank-Sum tests. The relationship between these measures and BMI, blood gas analysis (PaO2, PaCO2), or lung function (FEV1, FEV1/FVC, TLCO, TLC, RV) were evaluated by Pearson correlation analysis. Results Despite similar age, BMI and obstruction severity, women had a higher TLC and RV (median: TLC = 105%; RV = 166%) than men (median: TLC = 87%; RV = 132%). Adpl level was higher in women (median = 11,152 ng/ml) than in men (median = 10,239 ng/ml) and was negatively associated with hyperinflation (R = − 0,43) and hypercapnia (R = − 0,42). The proportion of the most active forms of Ad (HMW) was increased in hypoxemic women (median = 10%) compared with non-hypoxemic women (median = 8%) but was not modulated in men. Conclusion COPD pathophysiology seemed to be different in hypoxemic women and was associated to Ad modulations. Hyperinflation and air-trapping in association with hypercapnia and hypoxaemia, could contribute to a modulation of Adpl level and of its HMW forms. These results suggest the development of a distinct endotypic presentation, based on gender.

scholarly journals Chronic hypoxaemia and gender status modulate adiponectin pathway in severe COPD patients: new endotypic presentation?

2020 ◽  
Author(s):  
Mélany Pierard ◽  
Alexandra Tassin ◽  
Antoine Legrand ◽  
Alexandre Legrand
Keyword(s):  
Lung Function ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Lung Function Decline ◽  
Copd Patients ◽  
Gender Status ◽  
And Gender ◽  
The Impact ◽  
The Relationship ◽  
Gender Specific

Abstract Background: Disease progression in COPD patient is associated to lung function decline, leading to a higher risk of hypoxaemia and associated comorbidities, notably cardiovascular diseases (CV). Gender is also known to influence CV risk. Adiponectin (Ad), a cardio-protective hormone, was suggested as a biomarker for COPD risk management. However, determinants and consequences of Ad pathway modulation in COPD are unknown and gender specificities are poorly understood. We postulated that hypoxaemia and gender could influence Ad pathway and contribute to the appearance of a distinct endotype associated to an altered CV risk.Methods: The Ad plasmatic (Adpl) level and proportion of its different forms were evaluated in hypoxemic and non-hypoxemic COPD men or women. The relationship between these measures and BMI, blood gas analysis (PaO2, PaCO2), or lung function (FEV1, FEV1/FVC, TLCO, TLC, RV) were tested.Results: Despite similar age, BMI and obstruction severity, women had a higher TLC and RV than men. Adpl level was higher in women and negatively associated with hyperinflation and hypercapnia. The proportion of the most active forms of Ad (HMW) is increased in hypoxemic women but not in men. A positive correlation between TLCO and HMW form proportion was observed in hypoxemic men, whereas a negative correlation was detected in non-hypoxemic men.Conclusion: Physiopathology of COPD seems to be gender specific. Hypoxaemia, hypercapnia and hyperinflation are associated to gender-specific Ad pathway alterations. Given CV properties of Ad, the impact of such modulation on co-morbidities development have to be considered in future studies.

scholarly journals Can biomarkers of extracellular matrix remodelling and wound healing be used to identify high risk patients infected with SARS-CoV-2?: lessons learned from pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
D. J. Leeming ◽  
F. Genovese ◽  
J. M. B. Sand ◽  
D. G. K. Rasmussen ◽  
C. Christiansen ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Extracellular Matrix ◽  
Wound Healing ◽  
Lung Function ◽  
Pulmonary Fibrosis ◽  
Interstitial Lung Diseases ◽  
Lessons Learned ◽  
Lung Function Decline ◽  
Tissue Remodelling

AbstractPulmonary fibrosis has been identified as a main factor leading to pulmonary dysfunction and poor quality of life in post-recovery Severe Acute Respiratory Syndrome (SARS) survivor’s consequent to SARS-Cov-2 infection. Thus there is an urgent medical need for identification of readily available biomarkers that in patients with SARS-Cov-2 infection are able to; (1) identify patients in most need of medical care prior to admittance to an intensive care unit (ICU), and; (2) identify patients post-infection at risk of developing persistent fibrosis of lungs with subsequent impaired quality of life and increased morbidity and mortality. An intense amount of research have focused on wound healing and Extracellular Matrix (ECM) remodelling of the lungs related to lung function decline in pulmonary fibrosis (PF). A range of non-invasive serological biomarkers, reflecting tissue remodelling, and fibrosis have been shown to predict risk of acute exacerbations, lung function decline and mortality in PF and other interstitial lung diseases (Sand et al. in Respir Res 19:82, 2018). We suggest that lessons learned from such PF studies of the pathological processes leading to lung function decline could be used to better identify patients infected with SARS-Co-V2 at most risk of acute deterioration or persistent fibrotic damage of the lung and could consequently be used to guide treatment decisions.

Respiratory medicine

2018 ◽  
pp. 535-582
Author(s):  
Drew Provan
Keyword(s):  
Lung Function ◽  
Lung Volume ◽  
Needle Biopsy ◽  
Peak Flow ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Respiratory Medicine ◽  
Sweat Test ◽  
Volume Analysis ◽  
Flow Charts

Respiratory medicine is the study of lung function. This chapter outlines the investigations in current use, including blood gas analysis, sleepiness scans, bronchoscopy, flow–volume loops, peak flow charts, needle biopsy of the pleura, spirometry, lung volume analysis, and sweat test.

Thoracoabdominal restriction in supine men: CT and lung function measurements

1988 ◽  
Vol 64 (2) ◽  
pp. 599-604 ◽  
Author(s):  
L. Tokics ◽  
G. Hedenstierna ◽  
B. Brismar ◽  
A. Strandberg ◽  
H. Lundquist
Keyword(s):  
Lung Function ◽  
Lung Tissue ◽  
Total Lung Capacity ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Cross Sectional ◽  
Arterial Blood Gas ◽  
Lung Capacity ◽  
Arterial Blood ◽  
Volume Curve

Thoracoabdominal restriction was brought on by means of a corset, and the subsequent effects on thoracic dimensions and lung tissue were studied by computerized tomography (CT) and by various lung function tests in supine healthy volunteers (mean age 30 yr). Restriction caused reductions in total lung capacity (helium equilibration) from mean 6.84 to 4.80 liters, in functional residual capacity (FRC) from 2.65 to 2.08 liters, and in vital capacity from 5.16 to 3.45 liters. Closing capacity (single-breath N2 washout) fell from 2.42 to 1.88 liters, thus matching the reduction in FRC. The static pressure-lung volume curve was shifted to the right by 1.5 cmH2O at 50% of total lung capacity. However, no change in the slope of the curve was observed. The diaphragm was moved cranially by 1.2 cm, and the thoracic cross-sectional area was reduced by a mean 32 cm2 at a level just above the diaphragm. No changes in the lung tissue were seen on CT scanning. Gas exchange, as assessed by multiple inert gas elimination technique and arterial blood gas analysis, was unaffected by restriction. It is concluded that in supine subjects, thoracoabdominal restriction that reduces FRC by 0.6 liter is not accompanied by atelectasis (normal CT scan). In this respect the result differs from that found in anesthetized supine subjects who show the same fall in FRC and atelectasis in dependent lung regions.

scholarly journals The Effect of Optimized Ultrafiltration on Perioperative Pulmonary Function During Cardiopulmonary Bypass in Infants Under 10 kg

2021 ◽  
Vol 9 ◽  
Author(s):  
Jianhong Niu ◽  
Guangdi Zhai ◽  
Aibin Zheng ◽  
Juanying Zhou ◽  
Shengqi Jiang ◽  
...  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Treatment Group ◽  
Lung Function ◽  
Pulmonary Function ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Combination Method ◽  
Control Group ◽  
Acid Base Balance ◽  
Significant Difference

Objective: This study aims to investigate the effect of optimized ultrafiltration on perioperative electrolytes, acid–base balance, and pulmonary function during cardiopulmonary bypass (CPB) in infants with low body weight (under 10 kg), using traditional balanced ultrafiltration and modified ultrafiltration.Methods: A total of 30 children who underwent surgical correction for congenital heart disease in Changzhou Children's Hospital between January 2018 and December 2019 were randomly divided into two groups. In the treatment group, ultrafiltration pre-treatment was carried out with blood-containing priming fluid prior to CPB. Balanced ultrafiltration was performed during the operation, and optimized and modified ultrafiltration were conducted before closing and extubation. In the control group, traditional balanced ultrafiltration was used during the operation, and a modified ultrafiltration combination was used before closing and extubation. Indexes such as blood gas analysis and electrolytes were measured perioperatively, and pulmonary function was observed.Results: No deaths were reported in either group. The ventilator-assisted breathing time was shorter in the treatment group than in the control group (P < 0.05). The indexes of the treatment group were closer than those of the control group to the optimal physiological values. The concentrations of potassium ion (K+), lactate (Lac), and blood glucose (Glu) decreased, and there was significant difference between the two groups (P < 0.05) at the end of CPB. Hemoglobin (Hb) and hematocrit (HCT) in the treatment group increased (P < 0.01). Alveolar-arterial differences for oxygen (A-aDO2) and respiratory index (RI) increased significantly in both groups after operation. Children in the treatment group began to recover lung function earlier than children in the control group. Both A-aDO2 and RI were lower in the treatment group than in the control group at each time point after operation (P < 0.05).Conclusion: Optimizing and modifying the traditional ultrafiltration combination method can effectively shorten the ultrafiltration time, reduce the adverse impacts of the ultrafiltration technique, and improve the lung function of infants after operation.

Chronic fibrosing progressing interstitial lung disease: a decision of Multidisciplinary Expert Board

2021 ◽  
Vol 31 (4) ◽  
pp. 505-510
Author(s):  
S. N. Avdeev ◽  
S. Yu. Chikina ◽  
I. E. Tyurin ◽  
A. S. Belevskiy ◽  
S. A. Terpigorev ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Russian Federation ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Diagnosis And Treatment ◽  
Antifibrotic Therapy

Introduction. The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD).The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD.Results. According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs.Conclusion. Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.

scholarly journals Influence of dexamethasone and surfactant in the vitality and lung function of preterm lambs born by cesarean section

2019 ◽  
Vol 71 (1) ◽  
pp. 44-52
Author(s):  
F. Bovino ◽  
D.S. Denadai ◽  
L.G. Ávila ◽  
M. Deschk ◽  
P.S.P. Santos ◽  
...  
Keyword(s):  
Lung Function ◽  
Vital Signs ◽  
Blood Gas Analysis ◽  
Gas Analysis ◽  
Group Iv ◽  
High Pco2 ◽  
Control Group ◽  
Group Iii ◽  
Group I ◽  
Impaired Pulmonary Function

ABSTRACT The aim of this study was to assess the vitality and lung function of preterm lambs. Twenty seven preterm lambs were divided in four groups. Group I (n=6) preterm lambs/ control; group II (n=9) lambs born to mothers that were treated with dexamethasone antepartum; group III (n=6) lambs treated with surfactant; and group IV (n=6) lambs treated with surfactant and born to mothers that were treated with dexamethasone antepartum. The APGAR score was performed after birth (T0) and 15 minutes later (T1/4) to assess vitality. The vital signs, blood gas analysis, spirometry and capnometry were assessed immediately after birth and continued until 48 hours. Chest radiographs were performed at T0, T24 and T48. Significant rectal temperature interactions occurred at T1 and T6 depending on the type of treatment used. All animals showed low pH values, which were associated with high pCO2 values and HCO3 -values that increased over time from immediately after birth to two days of age. Higher tidal volume values were observed at T1/4, T1 and T24 when the animals were not treated with surfactant. Capnometry showed significant interactions between treatments at T0. Premature animals showed low vitality and impaired pulmonary function.

scholarly journals Potential of nintedanib in treatment of progressive fibrosing interstitial lung diseases

2019 ◽  
Vol 54 (3) ◽  
pp. 1900161 ◽  
Author(s):  
Lutz Wollin ◽  
Jörg H.W. Distler ◽  
Elizabeth F. Redente ◽  
David W.H. Riches ◽  
Susanne Stowasser ◽  
...  
Keyword(s):  
Lung Function ◽  
Tyrosine Kinases ◽  
Lung Diseases ◽  
Evidence Base ◽  
Interstitial Lung Diseases ◽  
Lung Pathology ◽  
Lung Function Decline ◽  
Approved Drugs

A proportion of patients with fibrosing interstitial lung diseases (ILDs) develop a progressive phenotype characterised by decline in lung function, worsening quality of life and early mortality. Other than idiopathic pulmonary fibrosis (IPF), there are no approved drugs for fibrosing ILDs and a poor evidence base to support current treatments. Fibrosing ILDs with a progressive phenotype show commonalities in clinical behaviour and in the pathogenic mechanisms that drive disease worsening. Nintedanib is an intracellular inhibitor of tyrosine kinases that has been approved for treatment of IPF and has recently been shown to reduce the rate of lung function decline in patients with ILD associated with systemic sclerosis (SSc-ILD). In vitro data demonstrate that nintedanib inhibits several steps in the initiation and progression of lung fibrosis, including the release of pro-inflammatory and pro-fibrotic mediators, migration and differentiation of fibrocytes and fibroblasts, and deposition of extracellular matrix. Nintedanib also inhibits the proliferation of vascular cells. Studies in animal models with features of fibrosing ILDs such as IPF, SSc-ILD, rheumatoid arthritis-ILD, hypersensitivity pneumonitis and silicosis demonstrate that nintedanib has anti-fibrotic activity irrespective of the trigger for the lung pathology. This suggests that nintedanib inhibits fundamental processes in the pathogenesis of fibrosis. A trial of nintedanib in patients with progressive fibrosing ILDs other than IPF (INBUILD) will report results in 2019.

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