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Connective Tissue Diseases and Lung Manifestations
Despite a number of prospective studies already initiated in the past years, the current epidemiology and course of interstitial lung disease (ILD) and pulmonary hypertension (PH) in patients with connective tissue disease (CTD) is still not well defined, particularly regarding its prevalence, incidence and the management of a broad spectrum of disease presentations. Major challenges include the identification of patients with progressive disease, the appropriate time point of therapeutic intervention and the underlying driver of disease (inflammatory or pro-fibrotic stimulus or both?). To address these issues in Western...
Continuation of Nintedanib After Single Lung Transplantation in IPF Subjects
The aim of this study is to assess the utility of nintedanib therapy in addition to usual transplant care in single lung transplant recipients with idiopathic pulmonary fibrosis (IPF). The investigators hypothesize that in IPF subjects who undergo single lung transplantation the administration of nintedanib 150 mg twice daily in addition to usual transplant care will result in better preservation of lung function at 24 months.
Coping Strategies Within Pulmonary Rehabilitation in Patients With IPF and COPD
The aim of this prospective observational trial is to evaluate the influence of Coping strategies on pulmonary rehabilitation outcomes like 6-minute walk distance and Quality of life.
Correlation Between Lung UltraSound Score and Hypoxemia for Interstitial Syndrome in Emergency Department
The primary objective of this study is to assess the presence of a correlation between the Lung ultrasound score (LUSS) and PaO2/FiO2 in patient presenting with interstitial syndrome (IS) in the ED. The primary end point considers the null hypothesis to be a negative linear distribution for LUSS and PaO2/FiO2 values. Secondary objectives and secondary end points One of the secondary objectives is to assess the correlation between the LUSS and PaCO2 in patient presenting with IS in the ED. The end point of this secondary outcome considers the null hypothesis to be a positive linear distribution for the LUSS and PaCO2 values. ...
Correlation Between the Change of Peripheral Lymphocyte Subsets and Clinically Amyopathic Dermatomyositis Combined With Rapidly Progressive Interstitial Lung Disease
Acute respiratory distress syndrome (ARDS) occurs in Clinically Amyopathic Dermatomyisitis(CADM) combined with Rapidly Progressive Interstitial Lung Disease(RPILD) within 1-3 months, which leads to death of patients and is difficult to treat. Even if high doses of glucocorticoids are ineffective, there is no recommended treatment for such patients, which is a huge medical challenge.Lymphopenia is an independent risk factor for death in CADM-RPILD, but the cause of lymphopenia is unclear.In this study, the level of lymphocyte subsets in peripheral blood was detected by flow cytometer, in order to further clarify the pathogenesis of...
Cortical Superficial Siderosis and Risk of Recurrent Intracerebral Hemorrhage in Cerebral Amyloid Angiopathy.
Cerebral amyloid angiopathy (CAA) is a major cause of lobar intracerebral hemorrhage (ICH) in the elderly with high risk of recurrence. The investigators aim to determine the relationship between cortical superficial siderosis (cSS), a MRI hemorrhagic marker of CAA and the risk of symptomatic ICH recurrence in a multicentric prospective cohort of patients with acute lobar ICH related to CAA. The investigators hypothesize that patients with cSS have an increased risk of recurrent symptomatic ICH relative to those without cSS.
Creation of a Biospecimen Repository From Patients With Interstitial Lung Diseases (ILD)
To develop a repository of blood samples from patients with ILD to support future studies into the development of such biomarkers. Patients with pneumonia and healthy patients will also be recruited as a control group.
Cyclophosphamide and Azathioprine vs Tacrolimus in Antisynthetase Syndrome-related Interstitial Lung Disease
"Antisynthetase syndrome (ASS) is one of the most severe inflammatory myopathy (IM), due to pulmonary involvement (interstitial lung disease, ILD). Until now, the most commonly used immunosuppresive therapy in Europe is Cyclophosphamide followed by different immunosuppressive drugs as maintenance therapy, including Azathioprine (and so called " European Strategy "). In the USA however, the first-line immunosuppressive treatment is Tacrolimus (so called " American Strategy "). None of these two different strategies has ever been studied prospectively, and there is no clear comparison of short and long-term treatment efficacy and...
Cyclosporine A in the TReatment of Interstitial Pneumonitis Associated With Sjogren's Syndrome
The purpose of this large multicenter, randomized, double-blinded, controlled clinical study is to investigate the efficacy and safety of Cyclosporin A for primary Sjogren's syndrome associated pneumonitis(pSS-IP), which has important implications for the establishment of standardized diagnosis and treatment of pSS-IP.
Deciphering Rheumatoid Arthritis-associated Interstitial Lung Disease Pathogenesis 2
The overall goal of this study is to identify risk and prognosis factors of Interstitial Lung Disease (ILD) in patients with rheumatoid arthritis (RA).