scholarly journals Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease

Respiration ◽  
2019 ◽  
Vol 99 (1) ◽  
pp. 73-82 ◽  
Author(s):  
Wim A. Wuyts ◽  
Marlies Wijsenbeek ◽  
Benjamin Bondue ◽  
Demosthenes Bouros ◽  
Paul Bresser ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Best Practice ◽  
Fibrotic Disease

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 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 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 Clinical course and management of idiopathic pulmonary fibrosis

2019 ◽  
Vol 14 ◽  
Author(s):  
Caitlin Quinn ◽  
Amy Wisse ◽  
Stephenie T. Manns
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Best Practice ◽  
Clinical Course ◽  
Early Stage ◽  
Supplemental Oxygen ◽  
Lung Function Decline ◽  
Eligibility Criteria ◽  
Obstructive Sleep ◽  
Antifibrotic Drugs

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (ILD) with an unpredictable clinical course. Although IPF is rare, healthcare professionals should consider IPF as a potential cause of unexplained chronic dyspnea and/or cough in middle-aged/elderly patients and refer patients to a pulmonologist for evaluation. Making a diagnosis of IPF requires specialist expertise. Multidisciplinary discussion, involving at minimum a pulmonologist and a radiologist with expertise in the differential diagnosis of ILDs, is required to ensure the most accurate diagnosis. Prompt diagnosis of IPF is important to enable patients to receive appropriate care from an early stage. Optimal management of IPF involves the use of antifibrotic drugs, as well as the provision of supportive care to alleviate symptoms and preserve patients’ quality of life. Antifibrotic drugs have been shown to slow lung function decline seen in patients with IPF. Patients’ symptoms and functional capacity can be improved through participation in pulmonary rehabilitation programs and the use of supplemental oxygen. Patient education is essential to help patients understand and manage their disease. The identification and management of comorbidities, such as obstructive sleep apnea, pulmonary hypertension, and emphysema, is also an important element of the overall care of patients with IPF. Patients with IPF should be evaluated for lung transplantation at an early stage to maximize their chances of meeting eligibility criteria. In this review, we describe the clinical course and impact of IPF and best practice in its management, highlighting the importance of taking a patient-centered approach.

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.

Role of Wnt/PCP pathway on bronchial cell function in Idiopathic Pulmonary Fibrosis

Pneumologie ◽  
2011 ◽  
Vol 65 (12) ◽  
Author(s):  
S Barkha ◽  
M Gegg ◽  
H Lickert ◽  
M Königshoff
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Cell Function

Regulation of Macroautophagy in Idiopathic Pulmonary Fibrosis

Pneumologie ◽  
2012 ◽  
Vol 66 (06) ◽  
Author(s):  
P Mahavadi ◽  
S Ahuja ◽  
I Henneke ◽  
W Klepetko ◽  
C Ruppert ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis
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