scholarly journals Idiopathic Pulmonary Fibrosis: Treatment and Prognosis

2015 ◽  
Vol 9s1 ◽  
pp. CCRPM.S23321 ◽  
Author(s):  
Hajime Fujimoto ◽  
Tetsu Kobayashi ◽  
Arata Azuma
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Treatment Options ◽  
Additional Treatment ◽  
Treatment Modalities ◽  
Epithelial Injury ◽  
Alveolar Epithelial ◽  
Alveolar Epithelial Injury ◽  
Long Term Oxygen Therapy

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a prognosis that can be worse than for many cancers. The initial stages of the condition were thought to mainly involve chronic inflammation; therefore, corticosteroids and other drugs that have anti-inflammatory and immunosuppressive actions were used. However, recently, agents targeting persistent fibrosis resulting from aberrant repair of alveolar epithelial injury have been in the spotlight. There has also been an increase in the number of available antifibrotic treatment options, starting with pirfenidone and nintedanib. These drugs prevent deterioration but do not improve IPF. Therefore, nonpharmacologic approaches such as long-term oxygen therapy, pulmonary rehabilitation, and lung transplantation must be considered as additional treatment modalities.

scholarly journals Epithelial–Mesenchymal Transition in the Pathogenesis of Idiopathic Pulmonary Fibrosis

Medicina ◽  
2019 ◽  
Vol 55 (4) ◽  
pp. 83 ◽  
Author(s):  
Francesco Salton ◽  
Maria Volpe ◽  
Marco Confalonieri
Keyword(s):  
Epithelial Cells ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Epithelial Mesenchymal Transition ◽  
Treatment Options ◽  
Alveolar Epithelium ◽  
Alveolar Epithelial Cells ◽  
Mesenchymal Transition ◽  
Alveolar Epithelial ◽  
Serious Disease

Idiopathic pulmonary fibrosis (IPF) is a serious disease of the lung, which leads to extensive parenchymal scarring and death from respiratory failure. The most accepted hypothesis for IPF pathogenesis relies on the inability of the alveolar epithelium to regenerate after injury. Alveolar epithelial cells become apoptotic and rare, fibroblasts/myofibroblasts accumulate and extracellular matrix (ECM) is deposited in response to the aberrant activation of several pathways that are physiologically implicated in alveologenesis and repair but also favor the creation of excessive fibrosis via different mechanisms, including epithelial–mesenchymal transition (EMT). EMT is a pathophysiological process in which epithelial cells lose part of their characteristics and markers, while gaining mesenchymal ones. A role for EMT in the pathogenesis of IPF has been widely hypothesized and indirectly demonstrated; however, precise definition of its mechanisms and relevance has been hindered by the lack of a reliable animal model and needs further studies. The overall available evidence conceptualizes EMT as an alternative cell and tissue normal regeneration, which could open the way to novel diagnostic and prognostic biomarkers, as well as to more effective treatment options.

scholarly journals Gastroesophageal Reflux and Idiopathic Pulmonary Fibrosis: A Review

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Ahmed Fahim ◽  
Michael Crooks ◽  
Simon P. Hart
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Gastroesophageal Reflux ◽  
Usual Interstitial Pneumonia ◽  
Lower Oesophageal Sphincter ◽  
Normal Lung ◽  
Alveolar Epithelial ◽  
Alveolar Epithelial Injury ◽  
Oesophageal Dysmotility ◽  
The Impact

The histological counterpart of idiopathic pulmonary fibrosis is usual interstitial pneumonia, in which areas of fibrosis of various ages are interspersed with normal lung. This pattern could be explained by repeated episodes of lung injury followed by abnormal wound healing responses. The cause of the initiating alveolar epithelial injury is unknown, but postulated mechanisms include immunological, microbial, or chemical injury, including aspirated gastric refluxate. Reflux is promoted by low basal pressure in the lower oesophageal sphincter and frequent relaxations, potentiated by hiatus hernia or oesophageal dysmotility. In susceptible individuals, repeated microaspiration of gastric refluxate may contribute to the pathogenesis of IPF. Microaspiration of nonacid or gaseous refluxate is poorly detected by current tests for gastroesophageal reflux which were developed for investigating oesophageal symptoms. Further studies using pharyngeal pH probes, high-resolution impedance manometry, and measurement of pepsin in the lung should clarify the impact of reflux and microaspiration in the pathogenesis of IPF.

Real-World Comprehensive Disease Management of Patients With Idiopathic Pulmonary Fibrosis

2019 ◽  
Vol 15 (1) ◽  
pp. 4-15 ◽  
Author(s):  
Wendi Mason ◽  
Sally McLaughlin ◽  
Sophy Dedopoulos ◽  
Erin Mahoney ◽  
Tonja Meadows ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Disease Progression ◽  
Pulmonary Rehabilitation ◽  
Poor Prognosis ◽  
Antifibrotic Therapy ◽  
Fibrotic Lung Disease ◽  
Long Term Oxygen Therapy

Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive, and fatal fibrotic lung disease with a poor prognosis. Antifibrotic therapy slows but does not halt disease progression. Patient education and management needs change during disease progression. Management is complicated by comorbidities, adverse events associated with antifibrotic therapy, and difficulties with long-term oxygen therapy and pulmonary rehabilitation. Treating IPF requires coordination between physicians and nurses in community and interstitial lung disease center settings. This review provides guidance for the healthcare professional who manages the essential aspects of care in IPF from diagnosis, through disease progression, and to the end of life.

scholarly journals Therapeutic Targeting of 15-PGDH in Murine Idiopathic Pulmonary Fibrosis

2019 ◽  
Author(s):  
Julianne N.P. Smith ◽  
Matthew D. Witkin ◽  
Alvin P. Jogasuria ◽  
Kelsey F. Christo ◽  
Thomas M. Raffay ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Mouse Model ◽  
Pulmonary Function ◽  
Small Molecule ◽  
Cell Types ◽  
Matrix Remodeling ◽  
Epithelial Injury ◽  
Lung Epithelial

AbstractIdiopathic pulmonary fibrosis (IPF) is a progressive disease characterized by interstitial remodeling and pulmonary dysfunction. The etiology of IPF is not completely understood but involves pathologic inflammation and subsequent failure to resolve fibrosis in response to epithelial injury. Therapeutic strategies for IPF are limited to anti-inflammatory and immunomodulatory agents, which are only partially effective. Prostaglandin E2 (PGE2) disrupts TGFβ signaling and suppresses myofibroblast differentiation, however practical strategies to raise tissue PGE2 during IPF have been limited. We previously described the discovery of a small molecule, (+)SW033291, that binds with high affinity to the PGE2-degrading enzyme 15-hydroxyprostaglandin dehydrogenase (15-PGDH) and increases PGE2 levels. Here we evaluated pulmonary 15-PGDH expression and activity and tested whether pharmacologic 15-PGDH inhibition (PGDHi) is protective in a mouse model of bleomycin-induced IPF. Long-term PGDHi was well-tolerated, reduced the severity of pulmonary fibrotic lesions and extracellular matrix remodeling, and improved pulmonary function in bleomycin-treated mice. Moreover, PGDHi attenuated both acute inflammation and weight loss, and decreased mortality. Endothelial cells and macrophages are likely targets as these cell types highly expressed 15-PGDH. In conclusion, PGDHi ameliorates inflammatory pathology and fibrosis in murine IPF, and may have clinical utility to treat human disease.Article summaryIn IPF, lung epithelial injury leads to local inflammation and fibrosis, which impairs pulmonary function. Inhibition of 15-PGDH using a well-tolerated small molecule attenuates inflammation and prevents pulmonary fibrosis and dysfunction in a mouse model of bleomycin-induced IPF.

Long-term nintedanib treatment in idiopathic pulmonary fibrosis (IPF): final data from INPULSIS-ON

2019 ◽  
Author(s):  
U Costabel ◽  
B Crestani ◽  
M Quaresma ◽  
M Kaye ◽  
T Ogura ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Final Data

scholarly journals Long-term treatment of patients with idiopathic pulmonary fibrosis with nintedanib: results from the TOMORROW trial and its open-label extension

Thorax ◽  
2017 ◽  
Vol 73 (6) ◽  
pp. 581-583 ◽  
Author(s):  
Luca Richeldi ◽  
Michael Kreuter ◽  
Moisés Selman ◽  
Bruno Crestani ◽  
Anne-Marie Kirsten ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Term Treatment ◽  
Adverse Event Profile ◽  
Open Label ◽  
Comparator Group ◽  
Long Term Treatment ◽  
Open Label Extension ◽  
Rate Of Decline

The TOMORROW trial of nintedanib comprised a randomised, placebo-controlled, 52-week period followed by a further blinded treatment period and an open-label extension. We assessed outcomes across these periods in patients randomised to nintedanib 150 mg twice daily or placebo at the start of TOMORROW. The annual rate of decline in FVC was −125.4 mL/year (95% CI −168.1 to −82.7) in the nintedanib group and −189.7 mL/year (95% CI −229.8 to −149.6) in the comparator group. The adverse event profile of nintedanib remained consistent throughout the studies. These results support a benefit of nintedanib on slowing progression of idiopathic pulmonary fibrosis beyond 52 weeks.

scholarly journals Molecular biomarkers in idiopathic pulmonary fibrosis

2014 ◽  
Vol 307 (9) ◽  
pp. L681-L691 ◽  
Author(s):  
Brett Ley ◽  
Kevin K. Brown ◽  
Harold R. Collard
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Alveolar Epithelial Cell ◽  
Underlying Disease ◽  
Current Approach ◽  
Molecular Biomarkers ◽  
Alveolar Epithelial ◽  
Cell Dysfunction ◽  
Collaborative Efforts ◽  
Intended Use

Molecular biomarkers are highly desired in idiopathic pulmonary fibrosis (IPF), where they hold the potential to elucidate underlying disease mechanisms, accelerated drug development, and advance clinical management. Currently, there are no molecular biomarkers in widespread clinical use for IPF, and the search for potential markers remains in its infancy. Proposed core mechanisms in the pathogenesis of IPF for which candidate markers have been offered include alveolar epithelial cell dysfunction, immune dysregulation, and fibrogenesis. Useful markers reflect important pathological pathways, are practically and accurately measured, have undergone extensive validation, and are an improvement upon the current approach for their intended use. The successful development of useful molecular biomarkers is a central challenge for the future of translational research in IPF and will require collaborative efforts among those parties invested in advancing the care of patients with IPF.

scholarly journals Long-term Effectiveness of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis Is Independent of Baseline Forced Vital Capacity

CHEST Journal ◽  
2016 ◽  
Vol 150 (4) ◽  
pp. 538A ◽  
Author(s):  
Paul Noble ◽  
Carlo Albera ◽  
Klaus-Uwe Kirchgaessler ◽  
Frank Gilberg ◽  
Ute Petzinger ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Forced Vital Capacity ◽  
Vital Capacity
Sign in / Sign up

Export Citation Format

Share Document