Genetic Causes and Clinical Features of Childhood Interstitial Lung Diseases in China
Interstitial Lung Diseases of ChildhoodGenetic TestingRecruitment of a carefully characterized cohort of chILD patients, to generate a database and biobank via collecting data on chILD in China. Importantly, compatibility with ongoing United States and Europe chILD data base developments will be factored in.
Families-At-risk for Interstitial Lung Disease Study
Interstitial Lung DiseaseIdiopathic Pulmonary FibrosisThe interstitial lung diseases (ILDs) are a family of closely related lung conditions characterized by alveolar inflammation, injury, and fibrosis not due to infection or neoplasia. While previously considered to be rare, a recent nationwide study found that idiopathic pulmonary fibrosis (IPF), a fibrotic ILD with a median survival of only 3.8 years, affects nearly 0.5% of older adults in the U.S. While pirfenidone and nintedanib slow the progression of IPF, neither reverses fibrosis nor prevents progression of the disease,and no studies to date have tested interventions that prevent the development of fibrotic ILDs.
Novel Markers of Disease Progression in Interstitial Lung Disease
Interstitial Lung DiseaseStudy design: A prospective cohort study with a longitudinal design, with several parameters measured serially at baseline and at pre-specified time points during three to five years of clinical observation. Primary objective: To validate quantitative analysis of lung sounds as a marker of disease progression in a cohort of patients with Interstitial Lung Disease (ILD) via correlation with the extent of fibrosis on HRCT, measured both via visual scoring and quantitative CT assessment, and other validated parameters of disease progression such as FVC, tolerance to exercise and patient-related outcomes (symptoms and quality of life). Secondary objectives: To determine the prognostic value of quantitative analysis of lung sounds and a series of novel putative biological markers, obtained from peripheral blood and bronchoalveolar lavage, toward poor outcomes (death, categorical decline of % predicted FVC >10%, acute exacerbation or respiratory-related hospitalisations) as compared to more conventional clinical, physiology and radiologic measurements.
An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE...
Pulmonary HypertensionInterstitial Lung Disease1 moreThis is a multicenter, open-label trial to evaluate the safety and efficacy of inhaled treprostinil in subjects with pre-capillary pulmonary hypertension (PH) associated with interstitial lung disease (ILD) including combined pulmonary fibrosis and emphysema (CPFE). The study will include about 266 patients who completed all required assessments in the RIN-PH-201 study at approximately 100 clinical trial centers. The study will continue Your participation in this study is voluntary and will last until you discontinue from the study or the study ends. The study will continue until each subject reaches the Week 108 visit or until inhaled treprostinil become commercially available for patients with PH associated with ILD including CPFE (whichever is sooner).
A Clinical Trial for CTD-ILD Treatment
Interstitial Lung DiseaseConnective Tissue DiseaseThe first objective of this protocol is to assess the tolerability and safety of N-acetylcysteine (NAC) in patients with connective tissue disease related interstitial lung disease (CTD-ILD).
DB289 Versus TMP-SMX for the Treatment of Acute Pneumocystis Jiroveci Pneumonia (PCP)
PneumoniaInterstitial Plasma Cell4 moreThe purpose of this study is to demonstrate the non-inferiority of pafuramidine maleate (DB289)versus trimethoprim-sulfamethoxazole (TMP-SMX)for the treatment of mild to moderately severe Pneumocystis pneumonia (PCP).
Study of Pomalidomide (CC-4047) to Evaluate Safety, Tolerability, Pharmacokinetics, Pharmacodynamics...
SclerodermaSystemic5 moreThe primary objective of this study is to evaluate the safety, tolerability, and efficacy of pomalidomide in the treatment of patients with systemic sclerosis with interstitial lung disease.
Safety of Anti-Depressant for Chronic Obstructive Pulmonary Disease (SAD-COPD)
COPDAnxiety3 moreObjective: Determine whether treatment of perceived stress, anxiety, and depression with anti-depressant therapy improves dyspnea scores, 6-minute walk (6MW) distance and quality of life (QoL) in patients with Chronic Obstructive Pulmonary Disease (COPD) and ILD (Interstitial Lung Disease) undergoing pulmonary rehab. The study is a prospective, randomized, double-blinded, placebo-controlled study to assess the effect of an SSRI on 6MW, dyspnea scores, and QoL in COPD and ILD patients undergoing pulmonary rehab. Thirty subjects that carry an ICD-9 code diagnosis of COPD and/or ILD and CES-D (Center for Epidemiologic Studies Depression) score of > 16 will be recruited from Duke Pulmonary Rehab. Multivariable regression models will be constructed to evaluate the relationship between perceived stress, anxiety, and depression with adjustments by race, gender, age, BMI and GOLD score. A multivariable regression model will be constructed to assess whether treatment of perceived stress, depression, and anxiety with antidepressant therapy (sertraline) is an effect modifier on 6MW distance and dyspnea scores in patients with COPD or ILD who are enrolled in pulmonary rehab. Descriptive statistics will be used to examine the socio-demographic characteristic data. Student t-tests will be performed to assess group differences in continuous data. Categorical variables will be examined using the Pearson's Chi-Squared test.
Functional Exercise Tests in Patients With Systemic Sclerosis
SclerodermaSystemic4 moreThe aim of the study is to examine the validity of peripheral oxygen saturation measurement during the 6-minute walk test in patients with Systemic Sclerosis (SSc) and to examine the utility of two other functional tests as markers of pulmonary involvement in patients with SSc.
Deciphering Rheumatoid Arthritis-associated Interstitial Lung Disease Pathogenesis 2
Rheumatoid ArthritisInterstitial Lung DiseaseThe overall goal of this study is to identify risk and prognosis factors of Interstitial Lung Disease (ILD) in patients with rheumatoid arthritis (RA).