scholarly journals The changing treatment landscape in idiopathic pulmonary fibrosis

2015 ◽  
Vol 24 (135) ◽  
pp. 65-68 ◽  
Author(s):  
Ulrich Costabel
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Median Survival ◽  
Fibrotic Disease ◽  
Long Time ◽  
Key Events ◽  
Made In

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible fibrotic disease of the lung that has greatly frustrated clinicians for a long time. The prognosis of IPF (median survival 2–5 years following diagnosis) is poorer than that of some cancers and for many years no significant advances were made in its management. However, between 2011 and 2014 a number of pivotal developments were made that have improved the outlook for patients with IPF. Herein, we review this rapidly changing landscape, discussing key events whilst still acknowledging that IPF remains a challenging disease to diagnose and manage.

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.

Tu1853 Idiopathic Pulmonary Fibrosis Is Associated With Increased Impedance Measures of Reflux Compared to Non-Fibrotic Disease Among Pre-Lung Transplant Patients

2014 ◽  
Vol 146 (5) ◽  
pp. S-855-S-856
Author(s):  
Sravanya Gavini ◽  
Raymond T. Finn ◽  
Wai-Kit Lo ◽  
Hilary J. Goldberg ◽  
Robert Burakoff ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Transplant ◽  
Fibrotic Disease ◽  
Transplant Patients

scholarly journals Probable usual interstitial pneumonia pattern on chest CT: is it sufficient for a diagnosis of idiopathic pulmonary fibrosis?

2020 ◽  
Vol 55 (4) ◽  
pp. 1802465 ◽  
Author(s):  
Jun Fukihara ◽  
Yasuhiro Kondoh ◽  
Kevin K. Brown ◽  
Tomoki Kimura ◽  
Kensuke Kataoka ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Survival Time ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Final Diagnosis ◽  
Chest Ct ◽  
Hazard Ratio ◽  
Adjusted Hazard Ratio ◽  
Made In

Recent studies have suggested that in patients with an idiopathic interstitial pneumonia (IIP), a probable usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient to diagnose idiopathic pulmonary fibrosis (IPF) without histopathology.We retrospectively compared the prognosis and time to first acute exacerbation (AE) in IIP patients with a UIP and a probable UIP pattern on initial chest CT.One hundred and sixty IIP patients with a UIP pattern and 242 with a probable UIP pattern were identified. Probable UIP pattern was independently associated with longer survival time (adjusted hazard ratio 0.713, 95% CI 0.536–0.950; p=0.021) and time to first AE (adjusted hazard ratio 0.580, 95% CI 0.389–0.866; p=0.008). In subjects with a probable UIP pattern who underwent surgical lung biopsy, the probability of a histopathological UIP pattern was 83%. After multidisciplinary discussion and the inclusion of longitudinal behaviour, a diagnosis of IPF was made in 66% of cases. In IPF patients, survival time and time to first AE were not associated with CT pattern. Among subjects with a probable UIP pattern, compared to non-IPF patients, survival time and time to first AE were shorter in IPF patients.In conclusion, IIP patients with a probable UIP pattern on initial chest CT had a better prognosis and longer time to first AE than those with a UIP pattern. However, when baseline data and longitudinal behaviour provided a final diagnosis of IPF, CT pattern was not associated with these outcomes. This suggests diagnostic heterogeneity among patients with a probable UIP pattern.

scholarly journals Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

2015 ◽  
Vol 9s1 ◽  
pp. CCRPM.S39897 ◽  
Author(s):  
Yutaro Nakamura ◽  
Takafumi Suda
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Fibrosis ◽  
Clinical Manifestations ◽  
Current Status ◽  
Acute Exacerbations ◽  
New Treatments ◽  
Disease Profiling ◽  
Parenchymal Lung Disease ◽  
Made In

Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF.

scholarly journals Pathologic Proteolytic Processing of N-Cadherin as a Marker of Human Fibrotic Disease

Cells ◽  
2022 ◽  
Vol 11 (1) ◽  
pp. 156
Author(s):  
Paul Durham Ferrell ◽  
Kristianne Michelle Oristian ◽  
Everett Cockrell ◽  
Salvatore Vincent Pizzo
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cell Surface ◽  
Cell Lines ◽  
Cell Adhesion Molecule ◽  
Protein Processing ◽  
Proteolytic Processing ◽  
Fibrotic Disease ◽  
Nonalcoholic Fatty Liver ◽  
Murine Antibody

Prior research has implicated the involvement of cell adhesion molecule N-cadherin in tissue fibrosis and remodeling. We hypothesize that anomalies in N-cadherin protein processing are involved in pathological fibrosis. Diseased tissues associated with fibrosis of the heart, lung, and liver were probed for the precursor form of N-cadherin, pro-N-cadherin (PNC), by immunohistochemistry and compared to healthy tissues. Myofibroblast cell lines were analyzed for cell surface pro-N-cadherin by flow cytometry and immunofluorescent microscopy. Soluble PNC products were immunoprecipitated from patient plasmas and an enzyme-linked immunoassay was developed for quantification. All fibrotic tissues examined show aberrant PNC localization. Cell surface PNC is expressed in myofibroblast cell lines isolated from cardiomyopathy and idiopathic pulmonary fibrosis but not on myofibroblasts isolated from healthy tissues. PNC is elevated in the plasma of patients with cardiomyopathy (p ≤ 0.0001), idiopathic pulmonary fibrosis (p ≤ 0.05), and nonalcoholic fatty liver disease with cirrhosis (p ≤ 0.05). Finally, we have humanized a murine antibody and demonstrate that it significantly inhibits migration of PNC expressing myofibroblasts. Collectively, the aberrant localization of PNC is observed in all fibrotic tissues examined in our study and our data suggest a role for cell surface PNC in the pathogenesis of fibrosis.

scholarly journals Cough in idiopathic pulmonary fibrosis

2016 ◽  
Vol 25 (141) ◽  
pp. 278-286 ◽  
Author(s):  
Mirjam J.G. van Manen ◽  
Surinder S. Birring ◽  
Carlo Vancheri ◽  
Vincent Cottin ◽  
Elisabetta A. Renzoni ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Chronic Cough ◽  
Future Developments ◽  
New Compounds ◽  
Insight Into ◽  
Made In ◽  
Gaining Insight

Many patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.

scholarly journals Failure of noninvasive prediction of pulmonary hypertension in patients with idiopathic pulmonary fibrosis

2015 ◽  
Vol 75 (3) ◽  
Author(s):  
F. Salajka ◽  
V. Bartoš ◽  
J. Novosad ◽  
J. Št'ásek ◽  
J. Bis ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Ultrasound Examination ◽  
Published Data ◽  
Noninvasive Method ◽  
Non Invasive ◽  
Invasive Method ◽  
The Mean ◽  
Made In

Background. Pulmonary hypertension (PH) in patients with advanced idiopathic pulmonary fibrosis (IPF) is a complication connected with unfavorable prognosis. Great efforts have been made in attempting to establish a reliable non-invasive method which would enable detection of this complication. In this context a formula using pulmonary function parameters was published with outstanding results. Methods. We tested the formula in 27 IPF patients who underwent a lung function examination, cardiac ultrasonography and catheterisation on the same day. Results. Pulmonary hypertension was detected by catheterisation in 17 patients (63%). In our group, contrary to the published data, the aforementioned formula was neither useful for detecting patients with a high probability of PH nor as a means of calculating the mean pulmonary artery pressure in individual patients (p=0.502 and p=0.833, respectively). Ultrasound examination reached borderline correlation with the values measured by catheterisation when we compare patients with relevant results (r=0.531, p=0.051). However, the examination gave no usable results in 13 patients (48%). Conclusion. Our data suggests that no reliable, noninvasive method is currently available for detecting and confirming PH in IPF patients. We did not confirm the usefulness of the published formula. Further carefully organised studies will be necessary to verify or refute it.

scholarly journals Greater cellular stiffness in fibroblasts from patients with idiopathic pulmonary fibrosis

2018 ◽  
Vol 315 (1) ◽  
pp. L59-L65 ◽  
Author(s):  
Jade Jaffar ◽  
Soung-Hee Yang ◽  
Sally Yunsun Kim ◽  
Hae-Won Kim ◽  
Alen Faiz ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Transforming Growth Factor ◽  
Transforming Growth Factor Β1 ◽  
Cellular Level ◽  
Lung Fibroblasts ◽  
Fibrotic Disease ◽  
Force Microscopy ◽  
Atomic Force ◽  
Organ Tissue

Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease involving degenerative breathing capacity. Fibrotic disease is driven by dysregulation in mechanical forces at the organ, tissue, and cellular level. While it is known that, in certain pathologies, diseased cells are stiffer than healthy cells, it is not known if fibroblasts derived from patients with IPF are stiffer than their normal counterparts. Using IPF patient-derived cell cultures, we measured the stiffness of individual lung fibroblasts via high-resolution force maps using atomic force microscopy. Fibroblasts from patients with IPF were stiffer and had an augmented cytoskeletal response to transforming growth factor-β1 compared with fibroblasts from donors without IPF. The results from this novel study indicate that the increased stiffness of lung fibroblasts of IPF patients may contribute to the increased rigidity of fibrotic lung tissue.

scholarly journals IDIOPATHIC PULMONARY FIBROSIS. CORRECTION OF ERRORS

2018 ◽  
Vol 99 (5) ◽  
pp. 269-278
Author(s):  
A. L. Yudin ◽  
Yu. A. Abovich ◽  
N. I. Afanas’eva
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Usual Interstitial Pneumonia ◽  
Concept Of Disease ◽  
Computer Tomographic ◽  
X Ray ◽  
Common Causes ◽  
Causes Of Errors ◽  
The Common ◽  
Made In

Substantial changes have been recently made in the concept of the diagnosis of idiopathic pulmonary fibrosis. The concept of disease pathogenesis and the pathomorphologic criteria for diagnosing usual interstitial pneumonia have been revised. All idiopathic pulmonary fibrosis treatments that existed prior to 2011 have been found to be ineffective. This article analyzes the currently existing computer tomographic criteria for the diagnosis and differential diagnosis of idiopathic pulmonary fibrosis and considers the common causes of errors and the possibilities of improving the existing X-ray criteria.

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