Efficacy and Safety of Pirfenidone in CTD-ILD
PirfenidoneConnective Tissue Diseases1 moreA single-center randomized controlled study will be used to observe the efficacy and safety of pirfenidone on CTD-ILD patients for 24 months. The main research endpoints is the lung function (FVC) at 6 months. The clinical dyspnea score, 6-minute walking distance, index of lung function and imaging indicators are evaluated, as well as primary disease activity and adverse reactions of therapy with glucocorticoid and immunosuppressants up to 24 months.
Tolerability and Safety of Nintedanib in Myositis Associated Interstitial Lung Disease: a Pilot...
Interstitial Lung DiseaseMyopathy1 moreThere is likely a role for using anti-fibrotic medications in patients with myositis-associated interstitial lung disease (MA-ILD) to slow down disease progression, especially in patients who have fibrotic and progressive disease. These patients however are currently being excluded from clinical trials of anti-fibrotic agents in progressive ILD because of the concomitant use of immunosuppression. The benefit of anti-fibrotic agents is being assessed in other rheumatic diseases and should be assessed in MA-ILD as well. They are a unique group of patients with a heterogeneous disease, and are much more frequently on concomitant immune-modulating therapy. As such, they should be studied on their own in separate clinical trials, and the use of nintedanib should be studied as an addition to standard of care immunosuppression. The objective of this study is to assess safety and tolerability of nintedanib in patients with MA-ILD.
Physiological Effects of HFNC During Exercise in Patients With Fibrosing Interstitial Lung Diseases...
Interstitial Lung DiseasePulmonary Rehabilitationin this study, the investigators aim to describe the effects of highflow nasal canulae oxygen therapy (HFNC) on respiratory muscle function during exercise in patients with fibrosing interstitial lung diseases (ILD) during an endurance test
Correlation Between PaO2/FiO2 and Lung Ultrasound Score in the Intensive Care Unit
Interstitial Lung DiseaseThe goal of this physiological interventional prospective study is to evaluate the improvement of the previously demonstrated correlation between PaO2/ FiO2 and Lung Ultrasound score (LUSS) in patients admitted in the ICU with an intesrtitial syndrom (IS) on the ultrasound of all aetiologies at inclusion and at twenty four and forty eight hours. The main question it aims to answer is if the LUSS is a valid tool to evaluate the severity of the IS Participants will initially have an arterial blood gas to evaluate the PaO2/FiO2 and in the ten minutes a lung ultrasound to evaluate the LUSS. This will be repeated at twenty four and forty eight hours.
Hyperpolarized 129Xe MRI for Imaging Pulmonary Function
Interstitial Lung DiseaseThe purpose of this study is to develop and evaluate the usefulness of MRI using 129Xe gas for regional assessment of pulmonary function. Specifically, three forms of 129Xe MRI contrast will be the investigators focus - 1) imaging of the 129Xe ventilation distribution, 2) imaging the alveolar microstructure via the 129Xe apparent diffusion coefficient (ADC), and 3) imaging 129Xe that dissolves in the pulmonary blood and tissues upon inhalation. Such imaging of 129Xe gas transfer is expected to be uniquely sensitive to pathologies affecting gas exchange (fibrosis, emphysema, pulmonary hypertension) and provide new insights regarding the normal resting heterogeneity of pulmonary gas exchange.
Use of cSVF Via IV Deployment for Residual Lung Damage After Symptomatic COVID-19 Infection
Pulmonary Alveolar ProteinosisCOPD4 moreCOVID-19 Viral Global Pandemic resulting in post-infection pulmonary damage, including Fibrotic Lung Disease due to inflammatory and reactive protein secretions damaging pulmonary alveolar structure and functionality. A short review includes: Early December, 2019 - A pneumonia of unknown cause was detected in Wuhan, China, and was reported to the World Health Organization (WHO) Country Office. January 30th, 2020 - The outbreak was declared a Public Health Emergency of International Concern. February 7th, 2020 - 34-year-old Ophthalmologist who first identified a SARS-like coronavirus) dies from the same virus. February 11th, 2020 - WHO announces a name for the new coronavirus disease: COVID-19. February 19th, 2020 - The U.S. has its first outbreak in a Seattle nursing home which were complicated with loss of lives.. March 11th, 2020 - WHO declares the virus a pandemic and in less than three months, from the time when this virus was first detected, the virus has spread across the entire planet with cases identified in every country including Greenland. March 21st, 2020 - Emerging Infectious Disease estimates the risk for death in Wuhan reached values as high as 12% in the epicenter of the epidemic and ≈1% in other, more mildly affected areas. The elevated death risk estimates are probably associated with a breakdown of the healthcare system, indicating that enhanced public health interventions, including social distancing and movement restrictions, should be implemented to bring the COVID-19 epidemic under control." March 21st 2020 -Much of the United States is currently under some form of self- or mandatory quarantine as testing abilities ramp up.. March 24th, 2020 - Hot spots are evolving and identified, particularly in the areas of New York-New Jersey, Washington, and California. Immediate attention is turned to testing, diagnosis, epidemiological containment, clinical trials for drug testing started, and work on a long-term vaccine started. The recovering patients are presenting with mild to severe lung impairment as a result of the viral attack on the alveolar and lung tissues. Clinically significant impairment of pulmonary function appears to be a permanent finding as a direct result of the interstitial lung damage and inflammatory changes that accompanied. This Phase 0, first-in-kind for humans, is use of autologous, cellular stromal vascular fraction (cSVF) deployed intravenously to examine the anti-inflammatory and structural potential to improve the residual, permanent damaged alveolar tissues of the lungs.
Fingolimod for the Abrogation of Interstitial Fibrosis and Tubular Atrophy Following Kidney Transplantation...
Interstitial FibrosisKidney Transplant; Complications6 moreThis is a randomized, double-blind, placebo-controlled trial in de novo kidney transplant patients to determine if the addition of fingolimod (brand name Gilenya®, candidate name- FTY720) on the background of standard immunosuppression will prevent expansion of the interstitial compartment of the transplanted kidney. Interstitial expansion is the precursor of interstitial fibrosis and graft loss. The study will test the hypothesis that abgrogating the fibrogenic effects of both the RhoA and mTOR pathways with fingolimod will reduce structural damage in transplanted kidneys and possible subsequent transplant failure.
FAPI PET for Lung Fibrosis
Interstitial Lung DiseaseIdiopathic Interstitial Pneumonias5 moreThis is a prospective exploratory biodistribution study in patients with interstitial lung disease (ILD). The purpose of this research study is to determine where and to which degree the FAPI tracer (68Ga-FAPI-46) accumulates in normal and fibrotic lung tissues of patients with interstitial lung disease. The study will include patients with interstitial lung disease who have or will initiate a new ILD medication OR will undergo tissue biopsy or surgery of the lung. The study will include 30 patients, the upper limit for PET imaging studies conducted under the Radioactive Drug Research Committee (RDRC) purview. Participants will be injected with up to 7 mCi of 68-GaFAPi and will undergo one PET/CT scan and one High Resolution CT of the lungs. The study is sponsored by Ahmanson Translational Theranostic Division at UCLA.
Senolytics Treatment of Interstitial Lung Disease in Common Variable Immunodeficiency
Common Variable ImmunodeficiencyInterstitial Lung Disease Due to Systemic DiseaseThe purpose of this study is to determine if the supplement, Fisetin, can be used as a treatment option for common variable immunodeficiency (CVID) by comparing its efficacy to placebo.
NIVATS Versus IGAVATS: a Pilot Trial Addressing Biopsy Quality in Undetermined Interstitial Lung...
Lung DiseasesInterstitialThe main objective of this study is to compare two independent groups of patients requiring lung biopsy for undetermined interstitial lung disease (ILD) in terms of diagnostic confidence as determined by histologists.