scholarly journals Gender differences at presentation of idiopathic pulmonary fibrosis in Sweden

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Dimitrios Kalafatis ◽  
Jing Gao ◽  
Ida Pesonen ◽  
Lisa Carlson ◽  
C. Magnus Sköld ◽  
...  

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a disease with poor prognosis mainly affecting males. Differences in clinical presentation between genders may be important both for the diagnostic work-up and for follow-up. In the present study, we therefore explored potential gender differences at presentation in a Swedish cohort of IPF-patients. Methods We studied patients included in the Swedish IPF- registry over a three-year period from its launch in 2014. A cross-sectional analysis was performed for data concerning demographics, lung function, 6- min walking test (6MWT) and quality of life (QoL) (King’s Brief Interstitial Lung Disease (K-BILD) score). Results Three hundred forty- eight patients (250 (72%) males, 98 (28%) females, median age 72 years in both genders) were included in the registry during the study period. Smoking history (N = 169 (68%) vs. N = 53 (54%), p < 0.05), baseline lung function (Forced vital capacity, % of predicted (FVC%): 68.9% ± 14.4 vs. 73.0% ± 17.7, p < 0.05; Total lung capacity, % of predicted (TLC%): 62.2% ± 11.8 vs. 68.6% ± 11.3%, p < 0.001) were significantly different at presentation between males and females, respectively. Comorbidities such as coronary artery disease (OR: 3.5–95% CI: 1.6–7.6) and other cardiovascular diseases (including atrial fibrillation and heart failure) (OR: 3.8–95% CI: 1.9–7.8) also showed significant differences between the genders. The K- BILD showed poor quality of life, but no difference was found between genders in total score (54 ± 11 vs. 54 ± 10, p = 0.61 in males vs. females, respectively). Conclusions This study shows that female patients with IPF have a more preserved lung function than males at inclusion, while males have a significant burden of cardiovascular comorbidities. However, QoL and results on the 6MWT did not differ between the groups. These gender differences may be of importance both at diagnosis and follow- up of patients with IPF.

2021 ◽  
Author(s):  
Yupeng Li ◽  
Shangwei Ning ◽  
Yi Yang ◽  
Hong Chen ◽  
Chen Wang

Abstract Background: Rapid advances in genetic and genomic technologies have begun to reshape our understanding of idiopathic pulmonary fibrosis (IPF). Ferroptosis, an iron-dependent form of regulated cell death, play an important role in the development of IPF. Therefore, our study aimed to explore the role of ferroptosis-related genes (FRGs) and their correlation with lung dysfunction and quality of life in patients with IPF. Methods: Datasets were acquired by researching the Gene Expression Omnibus. FRGs were acquired by researching GeneCard database and PubMed. Ferroptosis-related differentially expressed genes (FRDEGs) were identified according to integrating FRGs and the DEGs identified in the GSE110147 dataset. Candidate key genes were identified from the miRNA-target FRDEGs network and protein-protein interactions (PPI) network. The relationship between key genes and lung function or quality of life was calculated using the GSE32537 datasets.Results: 293 FRGs were obtained, and 71 FRDEGs were identified. According to enrichment analysis, cell growth and death and pathways associated cancer were the important pathways, and significant biological processes were mainly consisted of cellular responses to stimulus and various situations. In addition, this study constructed an PPI network and a miRNA-target network based on the 71 FRDEGs, determined 19 candidate key genes. Furthermore, acyl-CoA synthetase long chain family member 1 (ACSL1), integrin subunit beta 8 (ITGB8) and ceruloplasmin (CP) were identified as the key genes. The expression level of ACSL1 was the strongest predictor for lung function (negatively) including percent predicted forced vital capacity (FVC% predicted) and percent predicted diffusion capacity of the lung for carbon monoxide (Dlco% predicted) and quality of life (negatively). In addition, ITGB8 and CP were negatively associated with FVC% predicted. According to DrugBank and PubMed, 4 drugs and 16 drugs have been found to act on ACSL1 and CP, respectively. Conclusion: These results imply that FRGs may shed new understanding on disease mechanism and provide potential biomarkers and therapy target to predict IPF progression.


Author(s):  
Baruch Vainshelboim ◽  
Jonathan Myers

Resistance Training for Rehabilitation in Patients with Idiopathic Pulmonary Fibrosis Baruch Vainshelboim()1 and Jonathan Myers2  1Cardiovascular and Metabolic Disease Research Institute, Mountain View, CA, USA 2Cardiology Division, Veterans Affairs Palo Alto Health Care System / Stanford University, Palo Alto, CA, USA © The Authors   Abstract Idiopathic pulmonary fibrosis (IPF) is a debilitating condition that causes severe symptoms, impaired functional capacity and poor quality of life. Exercise training has been shown to be a safe and effective therapy for improving physical function, dyspnea and quality of life in patients with IPF. However, due to pathophysiological limitations and symptom burden, conducting safe and effective exercise interventions is challenging; optimal program components and training modalities are yet to be established. Resistance training (RT) is a well-established exercise modality for combating effects of aging, disuse and chronic diseases, although there are scarce data available among patients with IPF. The current review briefly summarizes the pathophysiology and clinical manifestations of IPF and describes the numerous health and clinical benefits of RT among older adults and patients with respiratory disease. It then explores the potential RT mechanisms for overcoming exercise limitations in IPF, which may provide a therapeutic opportunity for rehabilitation. Finally, the review suggests practical RT recommendations for pulmonary rehabilitation programs in patients with IPF.


2021 ◽  
Author(s):  
Yupeng Li ◽  
Shangwei Ning ◽  
Yi Yang ◽  
Hong Chen ◽  
Chen Wang ◽  
...  

Abstract Background: Rapid advances in genetic and genomic technologies have begun to reshape our understanding of idiopathic pulmonary fibrosis (IPF). Ferroptosis, an iron-dependent form of regulated cell death, play an important role in the development of IPF. Therefore, our study aimed to explore the role of ferroptosis-related genes (FRGs) and their correlation with lung dysfunction and quality of life in patients with IPF. Methods: Datasets were acquired by researching the Gene Expression Omnibus. FRGs were acquired by researching GeneCard database and PubMed. Ferroptosis-related differentially expressed genes (FRDEGs) were identified according to integrating FRGs and the DEGs identified in the GSE110147 dataset. Candidate key genes were identified from the miRNA-target FRDEGs network and protein-protein interactions (PPI) network. The relationship between key genes and lung function or quality of life was calculated using the GSE32537 datasets.Results: 293 FRGs were obtained, and 71 FRDEGs were identified. According to enrichment analysis, cell growth and death and pathways associated cancer were the important pathways, and significant biological processes were mainly consisted of cellular responses to stimulus and various situations. In addition, this study constructed an PPI network and a miRNA-target network based on the 71 FRDEGs, determined 19 candidate key genes. Furthermore, acyl-CoA synthetase long chain family member 1 (ACSL1), integrin subunit beta 8 (ITGB8) and ceruloplasmin (CP) were identified as the key genes. The expression level of ACSL1 was the strongest predictor for lung function (negatively) including percent predicted forced vital capacity (FVC% predicted) and percent predicted diffusion capacity of the lung for carbon monoxide (Dlco% predicted) and quality of life (negatively). In addition, ITGB8 and CP were negatively associated with FVC% predicted. According to DrugBank and PubMed, 4 drugs and 16 drugs have been found to act on ACSL1 and CP, respectively. Conclusion: These results imply that FRGs may shed new understanding on disease mechanism and provide potential biomarkers and therapy target to predict IPF progression.


2017 ◽  
Vol 3 (4) ◽  
pp. 00084-2017 ◽  
Author(s):  
Kaisa Rajala ◽  
Juho T. Lehto ◽  
Eva Sutinen ◽  
Hannu Kautiainen ◽  
Marjukka Myllärniemi ◽  
...  

This study was undertaken to investigate idiopathic pulmonary fibrosis (IPF) patients' health-related quality of life (HRQoL) and symptoms in a real-life cross-sectional study. Our secondary aim was to create a simple identification method for patients with increased need for palliative care by studying the relationship between modified Medical Research Council (mMRC) dyspnoea scale, HRQoL and symptoms.We sent a self-rating HRQoL questionnaire (RAND-36) and modified Edmonton Symptom Assessment Scale (ESAS) to 300 IPF patients; 84% of the patients responded to these questionnaires.The most prevalent (>80%) symptoms were tiredness, breathlessness, cough and pain in movement. An increasing mMRC score showed a linear relationship (p<0.001) to impaired HRQoL in all dimensions of RAND-36 and the severity of all symptoms in ESAS. Dimensions of RAND-36 fell below general population reference values in patients with mMRC score ≥2. The intensity of pain in movement (p<0.001) and at rest (p=0.041), and the prevalence of chest pain (p<0.001) had a positive linear relationship to increased mMRC score.An increasing mMRC score reflects impaired HRQoL and a high symptom burden. In clinical practice, the mMRC scale could be used for screening and identification of IPF patients with increased need for palliative care.


2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Kun Ji ◽  
Jianling Ma ◽  
Liangmin Wang ◽  
Niuniu Li ◽  
Shangjuan Dong ◽  
...  

Objective. To evaluate the efficacy and safety of traditional Chinese medicine (TCM) on lung function and quality of life of idiopathic pulmonary fibrosis (IPF) patients by meta-analysis. Methods. Randomized controlled trials (RCTs) related to TCM and IPF were searched on PubMed, EMBASE Cochrane Library, ClinicalTrials, China National Knowledge Infrastructure (CNKI), Wanfang Database, Chin VIP Information (VIP), and Chinese Biomedical Database (CBM) until December 2018. Standard mean difference (SMD) and 95% CI were calculated for the measurements related to lung function (FEV1/FVC, FVC%, FEV1%, TLC%, DLCO% or DLCO, and VC%) and other parameters (PO2, 6MWD, and SGRQ) when comparing TCM treatment to the control group. Relative risk (RR) and 95% CI of adverse events (AEs) were calculated to assess the safety of TCM. Results. A total of 40 RCTs comparing TCM to western medicine (WM) and involving 3194 IPF patients were eligible for the meta-analysis. The pooled results showed that TCM treatment improved significantly PO2 (SMD = 0.80, 95% CI 0.54 to 1.06, p<0.001), FEV1% (SMD = 0.57, 95% CI 0.42 to 0.71, p<0.001), DLCO% (SMD = 0.38, 95% CI 0.28 to 0.48, p<0.001), 6MWD (SMD = 0.70, 95% CI 0.56 to 0.84, p<0.001) and other measurements and reduced SGRQ scores (SMD = −0.51, 95% CI −0.70 to −0.22, p<0.001). Subgroup analysis of different study durations (3 months, ≥ 6 months) and comparison models (TCM vs. WM, TCM + WM vs. WM or TCM vs. placebo) showed similar results. No significant difference of risk of AEs was observed between both groups (RR = 0.66, 95% CI: 0.27–1.60, p=0.352). There was no obvious publication bias, and the pooled results were stable according to sensitivity analysis. Conclusion. To the best of our knowledge, the present study had the largest sample size. Our results indicated that TCM treatment may help provide benefit to the lung function, exercise capacity, and quality of life of IPF patients, alone or combined with WM, when compared to WM. More rigorous RCTs were needed in the future.


2019 ◽  
Vol 11 (12) ◽  
pp. 5547-5556 ◽  
Author(s):  
Nathan Hambly ◽  
Sarah Goodwin ◽  
Afia Aziz-Ur-Rehman ◽  
Nima Makhdami ◽  
Margaret Ainslie-Garcia ◽  
...  

2021 ◽  
Author(s):  
fares darawshy ◽  
Ayman Abu Rmeileh ◽  
Rottem Kuint ◽  
Dan Padawer ◽  
Khalil Karim ◽  
...  

Abstract RationaleSymptoms following acute COVID-19 infection are common, but their relationship to initial COVID-19 severity is unclear. We hypothesize that residual symptoms are related to disease severity, and severe acute COVID-19 infection is more likely to cause residual pulmonary damage. This study aims to evaluate symptoms, lung function and abnormal imaging within 3 months following COVID-19 infection, and whether they are related to initial disease severity.Methods A cross-sectional study was carried out at a designated post-COVID clinic in Hadassah Medical Center, Jerusalem, Israel. Patients with PCR-confirmed SARS-CoV-2 infection were evaluated within 12 weeks following infection and included both admitted and non-admitted subjects. All participants underwent assessment of symptoms, quality of life (SGRQ), pulmonary function tests, and imaging. Results A total of 208 patients (age 49.3±16 years) were included in the study. Initial disease severity was mild in 86, moderate in 49 and severe in 73 patients. At the time of follow up, there were no differences in frequency of residual symptoms or in SGRQ score between groups. Patients with severe COVID-19 were more likely to have residual dyspnea (p=0.04), lower oxygen saturation (p<0.01), lower FVC and TLC (p<0.001, p=0.03 respectively), abnormal CXR (p<0.01) and abnormal CT scan (p<0.01) compared to other groups. Conclusion Frequency of symptoms and impairment of quality of life at 12 weeks follow up are common and are not related to severity of initial COVID-19 disease. In contrast, reduced lung function and abnormal pulmonary imaging are more common in patients with more severe acute COVID-19 infection.


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