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Expert Review of Respiratory Medicine, 2007
Idiopathic pulmonary fibrosis (IPF; also known as cryptogenic fibrosing alveolitis) is a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with the histological pattern usual interstitial pneumonia (UIP). UIP is a distinct histological pattern observed in IPF but may also be found in other etiologies.
Joseph P, Lynch +4 more
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Idiopathic pulmonary fibrosis (IPF; also known as cryptogenic fibrosing alveolitis) is a distinctive type of chronic fibrosing interstitial pneumonia of unknown cause associated with the histological pattern usual interstitial pneumonia (UIP). UIP is a distinct histological pattern observed in IPF but may also be found in other etiologies.
Joseph P, Lynch +4 more
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Clinics in Chest Medicine, 1982
SUMMARY Idiopathic pulmonary fibrosis is a precise syndrome defined by clinical, roentgenographic, physiologic, and histopathologic features. Clinically, most patients have a nonproductive cough and dyspnea on exertion. The most common physical finding is the presence of crackles over the lower portions of both lungs; also, there may be clubbing ...
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SUMMARY Idiopathic pulmonary fibrosis is a precise syndrome defined by clinical, roentgenographic, physiologic, and histopathologic features. Clinically, most patients have a nonproductive cough and dyspnea on exertion. The most common physical finding is the presence of crackles over the lower portions of both lungs; also, there may be clubbing ...
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Current Opinion in Pulmonary Medicine, 1996
Idiopathic pulmonary fibrosis (IPF), or cryptogenic fibrosing alveolitis as it is known in the United Kingdom and Europe, is perhaps one of the most complex and frustrating pulmonary disorders. The cause of this illness is unknown. Treatment often founders in a mire of discontent, dissatisfaction, and failure. In fact, the philosophic homily "If you do
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Idiopathic pulmonary fibrosis (IPF), or cryptogenic fibrosing alveolitis as it is known in the United Kingdom and Europe, is perhaps one of the most complex and frustrating pulmonary disorders. The cause of this illness is unknown. Treatment often founders in a mire of discontent, dissatisfaction, and failure. In fact, the philosophic homily "If you do
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Annual Review of Medicine, 1993
Idiopathic pulmonary fibrosis kills half of its victims within five years of diagnosis. Currently available treatment regimens are disappointing, and the incidence of the disease appears to be increasing. Newer techniques of imaging coupled with laboratory advances in molecular and cellular biology may produce new strategies for modulating the disease ...
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Idiopathic pulmonary fibrosis kills half of its victims within five years of diagnosis. Currently available treatment regimens are disappointing, and the incidence of the disease appears to be increasing. Newer techniques of imaging coupled with laboratory advances in molecular and cellular biology may produce new strategies for modulating the disease ...
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Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis
Chest, 2007Idiopathic pulmonary fibrosis (IPF) is an untreatable diffuse parenchymal lung disease with a median survival of < 3 years. Pulmonary hypertension (PH) is frequently seen in patients with IPF and is commonly attributed to hypoxic vasoconstriction and capillary destruction. Pathology findings include endothelial proliferation and medial hypertrophy that
Nina M, Patel +3 more
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Nintedanib in idiopathic pulmonary fibrosis
Drugs of Today, 2015Idiopathic pulmonary fibrosis (IPF) conveys a median survival of 3 years and until recently has lacked effective therapies. Nintedanib, an orally available, small-molecule tyrosine kinase inhibitor with selectivity for vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF) receptors has ...
H V, Woodcock, T M, Maher
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Epigenetics in idiopathic pulmonary fibrosis
Biochemistry and Cell Biology, 2015Idiopathic pulmonary fibrosis (IPF) is a lethal chronic lung disorder with no effective treatment and a prognosis worse than that of lung cancer. Despite extensive research efforts, its etiology and pathogenesis still remain largely unknown. Current experimental evidence has shifted the disease paradigm from chronic inflammation towards the premise of
Argyrios, Tzouvelekis, Naftali, Kaminski
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Familial Idiopathic Pulmonary Fibrosis
2013Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown etiology, with an appearance of usual interstitial pneumonia on lung biopsy. To-date, about a 100 families diagnosed with IPF have been described. Familial IPF is defined as histologically confirmed IPF occurring in two or more members of a family.
K, Wytrychowski +2 more
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Treatments for Idiopathic Pulmonary Fibrosis
New England Journal of Medicine, 2014To the Editor: In reporting the results of the INPULSIS-1 and INPULSIS-2 trials, Richeldi et al. (May 29 issue) 1 describe the use of nintedanib in the treatment of idiopathic pulmonary fibrosis. In the same issue, King et al. 2 report the results of the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND ...
Shinyu, Izumi +2 more
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MUC5B and Idiopathic Pulmonary Fibrosis
Annals of the American Thoracic Society, 2015Abstract Idiopathic pulmonary fibrosis (IPF), a fatal disease that is a result of complex interactions between genetics and the environment, has limited treatment options. We have identified the MUC5B promoter polymorphism and other common genetic variants that in aggregate explain roughly one-third of disease risk. The MUC5B promoter
Ivana V, Yang +4 more
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