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The pathogenesis of idiopathic pulmonary fibrosis (IPF) is incompletely understood but recurrent epithelial injury occurs which evokes the coagulation cascade. Thrombin is produced as a result and is over expressed in IPF patients, so may be important in propagating disease activity. We aim to recruit patients with IPF and then complete FDG (18F-2-fluoro-2-deoxy-D-glucose fluorodeoxyglucose) PET (positron emission tomography) scans pre and post manipulation of the coagulation cascade to assess the role of this biological pathway in disease activity. Previous studies from our institution have demonstrated increased FDG avidity in the lungs of patients with IPF (assessed using FDG PET scans) but to date the cells and pathways responsible for this signal have not been identified and thus need further exploration.
The purpose of this study is to determine whether dasatinib and quercetin can reduce the expression of pro-inflammatory cells obtained by skin biopsy.
The investigators will conduct a single-center, prospective, randomized, placebo-controlled, double-blind pilot study of anti-herpesvirus therapy in patients with idiopathic pulmonary fibrosis (IPF). Patients with mild, moderate or severe IPF with serologic evidence of current or past Epstein-Barr Virus (EBV) or cytomegalovirus (CMV) infection. Randomization will be to pirfenidone plus placebo or pirfenidone plus valganciclovir. Thirty subjects will be enrolled and randomized to treatment with pirfenidone plus valganciclovir (20 subjects) or pirfenidone plus placebo (10 subjects) for 12 weeks. The primary outcome will be safety and tolerability will be determined by type, frequency and duration of adverse events (AEs) and serious adverse events (SAEs) after 12 weeks of study drug treatment. All study subjects will be offered bronchoscopy with bronchoalveolar lavage (BAL) at study initiation and upon completion of treatment (12 weeks). Subjects will then be followed up at routine clinic visits at 6, 9 and 12 months for data collection.
This study will evaluate whether regular yoga exercises designed specifically for patients with Idiopathic Pulmonary Fibrosis is associated with any change in quality of life. Half of the participants will be randomized to yoga, half to usual care. After the first group completes 12 weeks of yoga, the patients who were randomized to usual care will completed 12 weeks of yoga.
This study is a randomized, double-blind, placebo-controlled trial in which eligible IPF subjects will be randomized to receive GBT440 or Placebo orally daily.
Current diagnostic tools used in interstitial lung disease (ILD) do not meet the challenges set by the complex pathophysiology of this heterogenous group. The investigators therefore aimed to evaluate novel or not widely used diagnostic approaches for the detection and therapeutic monitoring of patients with various ILDs.
The Turkish Thoracic Society Usual Interstitial Pneumonia Registry (TURK-UIP) is a collaborative project to coordinate a team of investigators from various regions of Turkey. The purpose of the Registry is to collect epidemiological data on patients with idiopathic pulmonary fibrosis and other causes of UIP, and to obtain information about the natural course of the disease and the treatment response.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a high mortality. Health-related quality of life (HRQL) is impaired in patients with IPF. Little is known about the properties of recently developed HRQL questionnaires and about the longitudinal changes in HRQL, including factors with an impact on HRQL. Comorbidities have an impact on patients with IPF, but reports differ in incidence and prevalence. The impact of comorbidities on HRQL and disease progression has only been studied sparsely. Also, the association between biomarkers and disease progression need to be examined further.
The purpose of the present study is to evaluate the characteristics, management and clinical course of patients with IPF as treated under real-world in Italian Pulmonary Centres, in terms of symptoms, lung function and exercise tolerance during 12 months of observation.
Efficacy will be assessed by the change from baseline in St George's Respiratory Questionnaire (SGRQ) total score at week 12 and at week 24; by the change from baseline in dyspnea using the University of California San Diego Shortness of Breath Questionnaire (UCSC SOBQ) at week 12 and at week 24; whereas safety will be assessed by the percentage of patients with on-treatment Serious Adverse Events (SAE) from baseline to week 24.