Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung...
SclerodermaInterstitial Lung DiseaseScleroderma is a rare, long-term autoimmune disease in which normal tissue is replaced with dense, thick fibrous tissue. Normally, the immune system helps defend the body against disease and infection. In people with scleroderma, the immune system triggers fibroblast cells to produce too much of the protein collagen. The extra collagen becomes deposited in the skin and organs, causing hardening and thickening that is similar to the scarring process. Although scleroderma most often affects the skin, it also can affect other parts of the body, including the lungs, and in its most severe forms scleroderma can be life-threatening. Scleroderma-related interstitial lung disease is one example of a life-threatening scleroderma condition. In people with symptomatic scleroderma-related interstitial lung disease, scarring occurs in the delicate lung tissue, compromising lung function. The purpose of this study is to determine whether people with symptomatic scleroderma-related interstitial lung disease experience more respiratory benefits from treatment with a 2-year course of mycophenolate mofetil or treatment with a 1-year course of oral cyclophosphamide.
Rituximab Versus Cyclophosphamide in Connective Tissue Disease-ILD
Interstitial Lung DiseaseScleroderma2 moreInterstitial lung disease (ILD) is characterised by inflammation and scarring of the lung and is the leading cause of death in patients with systemic sclerosis, and contributes significantly to morbidity and mortality in many other connective tissue diseases (CTDs) such as polymyositis/dermatomyositis and mixed connective tissue disease. When ILD is extensive and/or progressive, immunosuppressive medication is often required to stabilize lung disease and alleviate symptoms. Current standard care for CTD associated ILD is extrapolated from studies performed in individuals with systemic sclerosis and comprises low dose corticosteroids and intravenous cyclophosphamide followed by oral azathioprine. In some individuals even this intensive immunosuppression is insufficient to prevent deterioration, and in a significant minority of affected individuals this results in respiratory failure and death. Rituximab has recently been reported as an effective 'rescue therapy' for stabilizing and even improving ILD in this patient group. Based on observations gained from this experience, the investigators believe that rituximab is a potential important alternative to current best therapy for this patient group. This study has therefore been initiated to evaluate the efficacy of rituximab (compared with standard therapy) in patients with progressive CTD related ILD.
INREAL - Nintedanib for Changes in Dyspnea and Cough in Patients Suffering From Chronic Fibrosing...
Lung DiseasesInterstitialThe primary objective of this observational study is to investigate the correlation between changes from baseline at 52 weeks in forced vital capacity (FVC) and changes from baseline at 52 weeks in dyspnea score points or cough score points as measured with the pulmonary fibrosis questionnaire (L-PF) questionnaire over 52 weeks of nintedanib treatment in patients suffering from chronic fibrosing interstitial lung disease (ILD) with a progressive phenotype (excluding idiopathic pulmonary fibrosis (IPF)).
Novartis Everolimus Transition
Graft DysfunctionInterstitial FibrosisTransition from tacrolimus based triple therapy with Mycophenolate Mofetil (MMF) and steroids in stable renal transplant patients to low intensity tacrolimus, everolimus and prednisone will be associated with improvement in Glomular Filtration Rate (GFR) and allograft fibrosis.
Effects of an Oxymizer on Endurance Time in Patients With Chronic Lung Diseases
COPDInterstitial Lung DiseaseThe Oxymizer pendant® is a special oxygen cannula that can be used to supply high flow long term oxygen therapy. It is compatible with a wide variety of oxygen sources. In a reservoir, the Oxymizer® stores pure oxygen so that the concentration of inhaled oxygen is increased. The aim of this study is to investigate the potential benefit of the Oxymizer® with regard to the blood oxygenation during exercise testing. Given that the "endurance time" is very sensitive for little changes in exercise capacity, this test will be used to show the difference between the Oxymizer® and a conventional nasal cannula regarding exercise duration at 70% of peak Watt and oxygenation. The tests will be accompanied by blood gas analysis, partial pressure of carbon dioxide and heart rate.
Progression of Renal Interstitial Fibrosis / Tubular Atrophy (IF/TA) According to Epithelial-mesenchymal...
Renal Interstitial FibrosisRecently, early biomarkers of renal interstitial fibrosis have been identified, amongst them de novo expression of vimentin by tubular epithelial cells, which is an intermediate filament, and the translocation of beta-catenin into their cytoplasm. These markers, when present, suggest that the epithelial cell undergoes a phenomenon well known as "epithelial to mesenchymal transition" (EMT) and could behaves like a myo-fibroblast. EMT is highly instrumental in several models of tissue fibrosis, including in the kidney. Actually, it has not only been demonstrated that these markers are detectable in the renal graft at an early time point post-transplant (i.e. as soon as three months), but also that the intensity of their expression correlates with the progression of interstitial fibrosis of the graft between 3 and 12 months
Opportunistic Screening for Osteoporotic Vertebral Fractures in Patients With Diffuse Interstitial...
OsteoporosisInterstitial Lung Disease1 moreIt is well known that some chronic respiratory pathologies such as asthma or chronic obstructive pulmonary disease (COPD) are associated with an increased risk of osteoporosis, due to the pathology itself and the therapies implemented (per-os or inhaled corticosteroids). Osteoporosis leads to an increased risk of fragility fracture, with an increased morbidity and mortality associated with severe fractures such as vertebral fractures. Also, osteoporotic vertebral fractures often occur at the thoracolumbar hinge, resulting in worsening of the thoracic kyphosis. However, to the best of our knowledge, the prevalence of osteoporotic vertebral fractures measured by CT scan in patients with interstitial lung disease (ILD) is not known. For these patients who already have impaired respiratory function, the appearance of vertebral fractures could impact their management and worsen their prognosis (additional restrictive syndrome, difficulties in analgesics management because of respiratory contraindications, difficulties in wearing a corset, etc...). In this context, it appears interesting to define the prevalence of osteoporosis and osteoporotic vertebral fractures at the thoracic spine and the thoraco-lumbar hinge in a population of patients followed for ILD. So, the main objective of this study is to describe the prevalence of vertebral osteoporotic fractures in an overall cohort of patients with ILD.
Palliation of Dyspnea With Morphine in Patients With Interstitial Lung Disease
Interstitial Lung DiseaseDyspnea1 more36 patients with interstitial lung disease will be randomized to 1 weeks treatment with morphine hydrochloride as oral linctus 5 mg, four times a day, and 5 mg as needed up to 4 times a day, or corresponding doses of placebo. VAS score for dyspnea will be evaluated after 1 hour and 1 week at follow up. Other questionnaires will also be evaluated (GAD-7, K-BUILD, Leicester score)
Hepatitis C Virus(HCV) Heart and Lung Study
Hepatitis CChronic5 moreThis is a multicenter study in Hepatitis C Virus (HCV) infected adult patients who also have advanced cardiac disease or advanced lung disease.
Rituxmab Versus IL-6 in Treating ILD
Interstitial Lung DiseaseSclerodermaAll SSc patients should know if they have been diagnosed with interstitial lung disease, or pulmonary hypertension and keep results from his/her last pulmonary function test, chest imaging, echocardiogram and stress test