scholarly journals Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Fabrizio Luppi ◽  
Meena Kalluri ◽  
Paola Faverio ◽  
Michael Kreuter ◽  
Giovanni Ferrara
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Epidemiological Studies ◽  
Elderly Subjects ◽  
Single Individual ◽  
Negative Consequences ◽  
Management Options ◽  
Artery Disease ◽  
Matrix Accumulation

AbstractIdiopathic pulmonary fibrosis (IPF) is a chronic and progressive disorder with an estimated median survival time of 3–5 years after diagnosis. This condition occurs primarily in elderly subjects, and epidemiological studies suggest that the main risk factors, ageing and exposure to cigarette smoke, are associated with both pulmonary and extrapulmonary comorbidities (defined as the occurrence of two or more disorders in a single individual). Ageing and senescence, through interactions with environmental factors, may contribute to the pathogenesis of IPF by various mechanisms, causing lung epithelium damage and increasing the resistance of myofibroblasts to apoptosis, eventually resulting in extracellular matrix accumulation and pulmonary fibrosis. As a paradigm, syndromes featuring short telomeres represent archetypal premature ageing syndromes and are often associated with pulmonary fibrosis. The pathophysiological features induced by ageing and senescence in patients with IPF may translate to pulmonary and extrapulmonary features, including emphysema, pulmonary hypertension, lung cancer, coronary artery disease, gastro-oesophageal reflux, diabetes mellitus and many other chronic diseases, which may lead to substantial negative consequences in terms of various outcome parameters in IPF. Therefore, the careful diagnosis and treatment of comorbidities may represent an outstanding chance to improve quality of life and survival, and it is necessary to contemplate all possible management options for IPF, including early identification and treatment of comorbidities.

scholarly journals Downregulation of exosomal let-7d and miR-16 in idiopathic pulmonary fibrosis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Donato Lacedonia ◽  
Giulia Scioscia ◽  
Piera Soccio ◽  
Massimo Conese ◽  
Lucia Catucci ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Therapy Response ◽  
Down Regulation ◽  
Cross Sectional ◽  
Exosomal Mirnas ◽  
Different Types ◽  
Sectional Analysis ◽  
Made In

Abstract Background Idiopathic Pulmonary Fibrosis (IPF) is a degenerative interstitial lung disease with both a poor prognosis and quality of life once the diagnosis is made. In the last decade many features of the disease have been investigated to better understand the pathological steps that lead to the onset of the disease and, moreover, different types of biomarkers have been tested to find valid diagnostic, prognostic and therapy response predictive ones. In the complexity of IPF, microRNA (miRNAs) biomarker investigation seems to be promising. Methods We analysed the expression of five exosomal miRNAs supposed to have a role in the pathogenesis of the disease from serum of a group of IPF patients (n = 61) and we compared it with the expression of the same miRNAs in a group of healthy controls (n = 15). Results In the current study what emerged is let-7d down-regulation and, unexpectedly, miR-16 significant down-regulation. Moreover, through a cross-sectional analysis, a clustering of the expression of miR-16, miR-21 and miR-26a was found. Conclusions These findings could help the individuation of previously unknown key players in the pathophysiology of IPF and, most interestingly, more specific targets for the development of effective medications.

scholarly journals Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

2018 ◽  
Vol 18 (1) ◽  
Author(s):  
K. Rajala ◽  
J. T. Lehto ◽  
E. Sutinen ◽  
H. Kautiainen ◽  
M. Myllärniemi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis

Impact of Pulmonary Rehabilitation on Exercise Tolerance and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis

2018 ◽  
Vol 38 (5) ◽  
pp. 273-278 ◽  
Author(s):  
Mansueto Gomes-Neto ◽  
Cassio Magalhães Silva ◽  
Diego Ezequiel ◽  
Cristiano Sena Conceição ◽  
Micheli Saquetto ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Rehabilitation ◽  
Exercise Tolerance

A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis

2010 ◽  
Author(s):  
Jeffrey J. Swigris ◽  
Sandra R. Wilson ◽  
Kathy E. Green ◽  
David B. Sprunger ◽  
Kevin K. Brown ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Assess Quality

scholarly journals Idiopathic pulmonary fibrosis: Educational needs of health-care providers, patients, and caregivers

2019 ◽  
Vol 16 ◽  
pp. 147997311985896 ◽  
Author(s):  
Deepa Ramadurai ◽  
Stephanie Corder ◽  
Tara Churney ◽  
Bridget Graney ◽  
Andrea Harshman ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Health Care Providers ◽  
Correct Diagnosis ◽  
Well Being ◽  
Poor Quality ◽  
Informational Needs ◽  
Care Providers ◽  
Perceived Needs

Idiopathic pulmonary fibrosis (IPF) is a progressive disease associated with poor quality of life. Debilitating symptoms and the reality of shortened survival impact patients’ physical and emotional well-being and constrain the lives of patients’ caregivers. This study assessed the informational needs of medical providers who care for patients with IPF, IPF patients themselves, and their caregivers. Tailored surveys were sent electronically to providers, patients with IPF, and caregivers of patients with IPF collected on a rolling basis in March of 2017. Providers answered questions regarding their own informational needs and what information they believed patients needed. Patients and caregivers identified their own informational needs and the perceived needs for each other. About 2636 surveys were sent to providers, including 2041 to physicians, of whom 156 completed it. One hundred sixty patients and 29 caregivers responded to the survey via a link on a website. Eighty-six percent of providers described themselves as physicians who diagnose and treat IPF patients themselves. Providers ranked information on “making the diagnosis of IPF” as their top informational need. Patients and caregivers chose “disease progression/what to expect” as the most important informational need for themselves and for each other. Providers want to make a correct diagnosis when IPF is in the differential diagnosis. Patients and caregivers desire clarity around how IPF will behave over time and what their futures with IPF will look like. Resources for patients and their caregivers should include information on disease natural history in empathically worded, clear, and easily accessible formats.

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 ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Gender Differences ◽  
Lung Function ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Poor Quality ◽  
Smoking History ◽  
Cross Sectional

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.

Burden of idiopathic pulmonary fibrosis on patients’ emotional well being and quality of life: a literature review

2020 ◽  
Vol 26 (5) ◽  
pp. 457-463 ◽  
Author(s):  
Katerina Antoniou ◽  
Apostolos Kamekis ◽  
Emmanouil K. Symvoulakis ◽  
Maria Kokosi ◽  
Jeffrey J. Swigris
Keyword(s):  
Quality Of Life ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Literature Review ◽  
Well Being
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