scholarly journals Idiopathic Pulmonary Fibrosis: New Insights to Functional Characteristics at Diagnosis

2014 ◽  
Vol 21 (3) ◽  
pp. e55-e60 ◽  
Author(s):  
Arturo Cortes-Telles ◽  
Lutz Forkert ◽  
Denis E O’Donnell ◽  
Onofre Morán-Mendoza
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Tertiary Care ◽  
Forced Expiratory Volume ◽  
Care Hospital ◽  
Severe Restriction ◽  
Alveolar Volume ◽  
Mild Restriction ◽  
Restrictive Pattern

BACKGROUND: The lung function of patients with idiopathic pulmonary fibrosis (IPF) has not been characterized in detail.OBJECTIVE: To characterize the heterogeneous physiological abnormalities that exist in patients with IPF during their initial clinical evaluation.METHODS: Lung function tests from 93 patients, performed within six months of the initial diagnosis of IPF, were obtained from a referral pulmonary function laboratory at a tertiary care hospital in Canada. A restrictive pattern was defined as total lung capacity (TLC) <95th percentile of predicted value. Patients with obstructive lung disease, lung cancer, emphysema and other restrictive lung diseases were excluded.RESULTS: On diagnosis, 73% of patients with IPF had a restrictive pattern, with a mean TLC of 72% of predicted. Mean forced vital capacity (FVC) was 71% and 44% of patients had an FVC <95th percentile. Mean diffusing capacity for carbon monoxide (DLco) was 60% and DLco/alveolar volume (VA) 92% of predicted. Increased severity of restriction – based on TLC – was associated with lower DLco(74% of predicted in mild restriction and 39% of predicted in severe restriction) and higher forced expiratory volume in 1 s (FEV1)/FVC ratio (82% of predicted in mild restriction and 90% of predicted in severe restriction) but not with age (76 years in mild restriction and 69 years in severe restriction). Regardless of severity of restriction, the average DLco/VA (≥86% of predicted) remained within normal limits.CONCLUSIONS: One in four patients with IPF had normal TLC and more than one-half had a normal FVC during initial evaluation. As the severity of the restriction increased, FEV1/FVC increased, DLcodecreased but DLco/VA remained normal.

Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis

Respiration ◽  
2020 ◽  
pp. 1-8
Author(s):  
Pierre-Henri Aussedat ◽  
Nader Chebib ◽  
Kais Ahmad ◽  
Jean-Charles Glerant ◽  
Gabrielle Drevet ◽  
...  
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Lung Biopsy ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Surgical Lung Biopsy ◽  
Before And After ◽  
The Impact

<b><i>Background:</i></b> Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). <b><i>Objectives:</i></b> The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. <b><i>Methods:</i></b> This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. <b><i>Results:</i></b> In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (<i>p</i> &#x3c; 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC &#x3c;50% at baseline. <b><i>Conclusion:</i></b> In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. <b><i>Summary at a Glance:</i></b> This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

scholarly journals Management of Idiopathic Pulmonary Fibrosis Cases at a Tertiary Care Hospital

2019 ◽  
Vol 2 (2) ◽  
pp. 90-95
Author(s):  
Mahesh N ◽  
◽  
Sadanand C D ◽  
Durga Lawande ◽  
◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Tertiary Care Hospital ◽  
Tertiary Care ◽  
Care Hospital

Fibrosis pulmonar idiopática: Impacto de la biopsia de pulmón en la función pulmonar

2021 ◽  
Vol 3 (1) ◽  
pp. 18-19
Author(s):  
Ricardo Lemus-Rangel
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Lung Biopsy ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Surgical Lung Biopsy ◽  
Before And After ◽  
The Impact

<b>Background:</b> Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). <b>Objectives:</b> The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. <b>Methods:</b> This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV<sub>1</sub>), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. <b>Results:</b> In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (<i>p</i> &#x3c; 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV<sub>1</sub>, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC &#x3c;50% at baseline. <b>Conclusion:</b> In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. Summary at a Glance: This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

scholarly journals A STUDY OF PULMONARY FUNCTION TESTS IN A PATIENTS WITH TYPE 2 DIABETES MELLITUS AND THEIR ASSOCIATION WITH GLYCAEMIC CONTROL AND DURATION OF DIABETES AT TERTIARY CARE HOSPITAL

2019 ◽  
Vol 3 (11) ◽  
Author(s):  
Dr. Sonam Gupta ◽  
Dr. Yadvendra Gupta
Keyword(s):  
Lung Function ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Tertiary Care ◽  
Care Hospital ◽  
Duration Of Diabetes ◽  
Chronic Hyperglycemia ◽  
Function Tests ◽  
Restrictive Pattern

Background- The pulmonary and other late complications of diabetes share a similar microangiopathy mechanism. Since, they share common mechanisms there may be associations between lung function and markers of microangiopathy. Therefore, we aimed to determine the association of pulmonary function with diabetes and the correlation of pulmonary function abnormalities with microvascular complications. Methods- This was a cross sectional comparative study carried out in a tertiary care teaching hospital in Jaipur,Rajasthan, India. Eligible participants included 100 adults below 60 years of age with T2DM attending the outpatient clinics or admitted to the wards during the study period, and an equal number of non-diabetic adults matched for age and gender who served as the comparative controls. Results- The present study clearly showed a highly statistically significant p value when the lung function tests (FVC, FEV1, and PEFR) were compared between type 2 diabetics and age, sex, BMI matched controls. The duration of DM and PFT correlation was found stastically significant and HbA1c and PFT correlation was found stastically Insignificant. Conclusion- Chronic hyperglycemia in diabetes may lead to diabetes associated systemic inflammation which results in airway and lung damage. Diabetes was more common in the sixth decade of life with slight male preponderance. The most common PFT derangement pattern in diabetic subjects is a restrictive pattern and having a significantly longer duration of a diabetic. The short-term indicators of glycemic controls were not significantly associated with a restrictive pattern of PFT. Keywords: Diabetes, Pulmonary function test, HbA1c, Duration of diabetes.

scholarly journals Gene correlation network analysis to identify regulatory factors in idiopathic pulmonary fibrosis

Thorax ◽  
2018 ◽  
Vol 74 (2) ◽  
pp. 132-140 ◽  
Author(s):  
John E McDonough ◽  
Naftali Kaminski ◽  
Bernard Thienpont ◽  
James C Hogg ◽  
Bart M Vanaudenaerde ◽  
...  
Keyword(s):  
Gene Expression ◽  
Transcription Factors ◽  
Network Analysis ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Peripheral Blood ◽  
Underlying Disease ◽  
Forced Expiratory Volume ◽  
Correlation Network

BackgroundIdiopathic pulmonary fibrosis (IPF) is a severe lung disease characterised by extensive pathological changes. The objective for this study was to identify the gene network and regulators underlying disease pathology in IPF and its association with lung function.MethodsLung Tissue Research Consortium dataset with 262 IPF and control subjects (GSE47460) was randomly divided into two non-overlapping groups for cross-validated differential gene expression analysis. Consensus weighted gene coexpression network analysis identified overlapping coexpressed gene modules between both IPF groups. Modules were correlated with lung function (diffusion capacity, DLCO; forced expiratory volume in 1 s, FEV1; forced vital capacity, FVC) and enrichment analyses used to identify biological function and transcription factors. Module correlation with miRNA data (GSE72967) identified associated regulators. Clinical relevance in IPF was assessed in a peripheral blood gene expression dataset (GSE93606) to identify modules related to survival.ResultsCorrelation network analysis identified 16 modules in IPF. Upregulated modules were associated with cilia, DNA replication and repair, contractile fibres, B-cell and unfolded protein response, and extracellular matrix. Downregulated modules were associated with blood vessels, T-cell and interferon responses, leucocyte activation and degranulation, surfactant metabolism, and cellular metabolic and catabolic processes. Lung function correlated with nine modules (eight with DLCO, five with FVC). Intermodular network of transcription factors and miRNA showed clustering of fibrosis, immune response and contractile modules. The cilia-associated module was able to predict survival (p=0.0097) in an independent peripheral blood IPF cohort.ConclusionsWe identified a correlation gene expression network with associated regulators in IPF that provides novel insight into the pathological process of this disease.

scholarly journals Functional parameters of small airways can guide bronchodilator use in idiopathic pulmonary fibrosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Po-Wei Hu ◽  
Hsin-Kuo Ko ◽  
Kang-Cheng Su ◽  
Jia-Yih Feng ◽  
Wei-Juin Su ◽  
...  
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Forced Expiratory Volume ◽  
Airflow Limitation ◽  
Small Airways ◽  
Symptom Scores ◽  
Bronchodilator Treatment ◽  
Long Acting

Abstract Idiopathic pulmonary fibrosis (IPF) may present comorbid obstructive lung diseases with small airway dysfunction (SAD). Existing guidelines suggest that inhaled bronchodilators should be used if the ratio of forced expiratory volume in the 1st second and forced vital capacity (FEV1/FVC) < 0.7 in IPF. However, most IPF patients have FEV1/FVC > 0.7 even with coexisting emphysema. We retrospectively enrolled IPF patients who were registered at our outpatient clinic. At baseline, 63 patients completed computed tomography (CT) scans, lung function measurements, and symptom questionnaires. Among these patients, 54 (85.71%) underwent antifibrotic treatment and 38 (60.32%) underwent long-acting bronchodilator treatment. The median FEV1/FVC was 0.86. Not all patients treated with bronchodilators showed significant changes in lung function. IPF patients with SAD, determined by IOS parameters, showed significant improvement in FEV1, FEF25–75%, and symptom scores after bronchodilator treatment. Bronchodilator efficacy was not observed in patients without SAD. CT-confirmed emphysema was seen in 34.92% of patients. There were no changes in lung function or symptom scores after bronchodilator treatment in patients with emphysema. In conclusion, FEV1/FVC cannot reflect the airflow limitation in IPF. Emphysema in IPF is not a deciding factor in whether patients should receive bronchodilator treatment. IOS parameters may be useful to guide bronchodilator therapy in patients with IPF coexisting with SAD.

scholarly journals Effectiveness of pirfenidone in idiopathic pulmonary fibrosis according to the autoantibody status: a retrospective cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Myung Jin Song ◽  
Sang Hoon Lee ◽  
Ji Ye Jung ◽  
Young Ae Kang ◽  
Moo Suk Park ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Vital Capacity ◽  
Tertiary Care ◽  
Care Hospital ◽  
Diffusion Capacity ◽  
Autoantibody Status ◽  
The Mean ◽  
One Year ◽  
Positive Groups

Abstract Background Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear. Methods We retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies. Results We included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively. Conclusions Reductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.

Analysis of patients with Idiopathic Pulmonary Fibrosis (IPF) with more severe lung function impairment treated with pirfenidone in RECAP

Pneumologie ◽  
2018 ◽  
Vol 72 (S 01) ◽  
pp. S47-S48
Author(s):  
U Costabel ◽  
C Albera ◽  
KU Kirchgaessler ◽  
F Gilberg ◽  
U Petzinger ◽  
...  
Keyword(s):  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Function Impairment ◽  
Lung Function Impairment ◽  
Severe Lung

scholarly journals Chemotherapy in idiopathic pulmonary fibrosis and small-cell lung cancer with poor lung function

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiyue Zhang ◽  
Wei Li ◽  
Chunyan Li ◽  
Jie Zhang ◽  
Zhenzhong Su
Keyword(s):  
Lung Cancer ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Small Cell Lung Cancer ◽  
Cell Lung Cancer ◽  
Small Cell ◽  
Small Cell Lung ◽  
High Resolution Computed Tomography ◽  
Chemotherapeutic Regimen

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.

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