Immunopathologic Profiles and Blood Biomarkers in Patients With IPF
Idiopathic Pulmonary FibrosisExamination of expression of PD-L1, PD-L2, Beta- catenin, B-cell follicles and Tenascin- C in patients with IPF compared with other interstitial lung diseases. Examination of anti HSP 70, p-ANCA, c-ANCA, CD4+/CD28- and CD8+/CD28- cells in patients with IPF compared with other interstitial lung diseases. Compare the above mentioned findings with changes in pulmonary function tests, 6 minute walking test, exacerbation and mortality over a 2 year follow-up period.
Microaspiration in Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisHypothesis 1: Microaspiration, as diagnosed by bronchoalveolar lavage (BAL) pepsin, is common in patients with IPF. Hypothesis 2a: Baseline clinical variables and co-morbid conditions are risk factors for microaspiration in patients with IPF. Hypothesis 2b: Baseline biological variables reflecting alveolar epithelial injury and inflammation are markers of microaspiration in IPF. Hypothesis 3a: Microaspiration will lead to a more rapid rate of decline in pulmonary function. Hypothesis 3b: Microaspiration will lead to higher rates of urgent medical care use (i.e. unscheduled clinic visit, emergency room visit, or hospitalization).
Idiopathic Pulmonary Fibrosis Registry for Future Studies
Lung DiseaseInterstitial1 moreTo establish a registry of patients for future studies of Inflammation in Interstitial Lung Disease/Idiopathic Pulmonary Fibrosis.
Herpesvirus in Idiopathic Pulmonary Fibrosis
Lung DiseasesHerpesvirus InfectionsThe purpose of this study is to: 1) to develop a method to quantify Epstein Barr Virus (EBV) load in lung tissue of humans and to determine whether EBV viral load is significantly higher in lung tissue from patients with idiopathic pulmonary fibrosis (IPF) than in control lung tissue; 2) to determine whether EBV localized to epithelial cells in IPF lungs and to relate epithelial positivity to tissue viral load; 3) to measure viral load in induced sputum from IPF subjects over time in order to determine whether periodic active herpes virus replication occurred in the respiratory tract; and 4) to compare longitudinal measures of viral load in induced sputum with simultaneously collected saliva in order to assess the clinical utility of the two approaches.
Lung Disease Associated With Rheumatoid Arthritis
Pulmonary FibrosisPulmonary fibrosis (PF) is a condition in which the lungs of a patient become scarred and fibrous. It has been known to occur in as many as 40% of patients diagnosed with rheumatoid arthritis (RA). The cause of the pulmonary fibrosis in patients with RA is unknown. Patients participating in this study will undergo a series of tests and examinations before and throughout the study. The tests include blood and urine tests, electrical measures of heart function (ECG), chest x-rays, CAT scans, nuclear medicine scans, breathing tests, exercise tests, and fiberoptic bronchoscopy. The goals of this study are to: Estimate how common pulmonary fibrosis is in patients with rheumatoid arthritis, Describe the natural course of pulmonary fibrosis in patients with rheumatoid arthritis, Estimate the survival rate of patients with pulmonary fibrosis and rheumatoid arthritis, and Learn more about the factors that contribute to the development or progression fibrotic lung disease....
Segmental Bronchoalveolar Lavage
AsthmaPulmonary FibrosisBronchoalveolar lavage (BAL) is a diagnostic and therapeutic procedure conducted by placing a small fiberoptic scope into the lung of a patient, and injecting sterile water (saline) into the lung and removing the fluid. The sterile solution removed contains secretions, cells, and protein from the lower respiratory tract. This sample can be analyzed to provide more information about possible disease processes going on in the lungs. This protocol will be used to perform BAL, bronchial brushing, and bronchial wall biopsy in normal volunteers and patients with pulmonary disease. The samples collected during the study will be used to examine biochemical processes in the lung that may contribute to lung disease
Estimated Impact of Fungal Colonization in Cystic Fibrosis From Secondary Exploitation of MucoFong...
Cystic Fibrosis LungThe main objective of the project "MucoFong" (19021906 national french program n which Vaincre La Mucoviscidose participated: N82006/ 351) was to determine the fungi present the respiratory tract of CF patients responsible for either colonization or authentic infectious diseases. The Mucofong data allowed the team to provide for the 1st time national French guidelines for the management of CF sputum mycological analysis (MucoMicrobes work group coordinated by Prof. Plésiat published in 2015 in the REMIC book). Nevertheless, the team has a comprehensive database that it still has to analyze beyond these initial results. The main goal today is to clarify the role of fungi in the lung function degradation of these patients by studying the overall risk and estimated impact of fungal colonization in our cohort.
Determinants of Early Cystic Fibrosis Lung Disease
Cystic Fibrosis in ChildrenThe overall objective of this study is to determine the impact early nutritional and respiratory indices have on early CF lung disease. This knowledge will guide clinical management of infants with CF, who are now primarily diagnosed through newborn screening.
Assessment of Skeletal Muscle Dysfunction in Patient With Idiopathic Pulmonary Fibrosis
Interstitial Lung DiseaseSkeletal Muscle DysfunctionInterstitial lung disease includes a heterogeneous group of chronic lung conditions that is characterized by exertional dyspnoea and poor health related quality of life . includes idiopathic pulmonary fibrosis of unknown cause And another groups are caused by occupational, inorganic or organic exposure, drug- induced toxicities, or are secondaries to connective tissue disease The clinical course and outcome of interstitial lung diseases are highly variable between different sub types, but survival after diagnosis of idiopathic pulmonary fibrosis is only 2.5 to 5 years is a progressive and fibrosing lung disease that is characterized by architectural distortion of the lung parenchyma and is progressive, with a dismal prognosis Also patient with idiopathic pulmonary fibrosis generally demonstrate greater abnormalities of exercise induced gas exchange than those with other forms of Interstitial lung disease
A Longitudinal Study of Hermansky-Pudlak Syndrome Pulmonary Fibrosis
Hermansky Pudlak SyndromeHermansky-Pudlak Syndrome (HPS) is a rare genetic disease that is associated with oculocutaneous albinism, bleeding, granulomatous colitis, and pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. Pulmonary fibrosis causes shortness of breath and progressive decline in lung function. In HPS patients with at-risk subtypes, almost all adults eventually develop fatal pulmonary fibrosis unless they undergo lung transplantation. The purpose of this study is to identify the earliest measurable pulmonary disease activity in individuals at-risk for HPS pulmonary fibrosis. The study also aims to develop biomarkers that will aid in understanding of the causes of HPS pulmonary fibrosis and facilitate more rapid conduct of therapeutic trials in HPS patients with mild pulmonary disease in the future.