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Abatacept for the Treatment of Myositis-associated Interstitial Lung Disease
A randomized, controlled pilot trial to evaluate the efficacy and safety of subcutaneous Abatacept in treating interstitial lung disease associated with the anti-synthetase syndrome.
18 Years and OverLearn More -
Abatacept for the Treatment of Relapsing, Non-Severe, Granulomatosis With Polyangiitis (Wegener's)
Multi-center, randomized, double-blind, placebo-controlled trial to evaluate the efficacy of abatacept to achieve sustained glucocorticoid-free remission in patients with relapsing non-severe granulomatosis with polyangiitis (Wegener's) (GPA) . Participants will be randomized 1:1 to receive either abatacept 125 mg or placebo administered by subcutaneous injection once a week. Participants will continue on study treatment for a minimum of 12 months unless they experience a disease relapse or disease flare. Participants who experience a non-severe disease relapse, non-severe disease worsening, or who have not achieved remission by month 6...
15 Years and OverLearn More -
A Clinical Evaluation of Baofeikang Granule in Combined Pulmonary Fibrosis and Emphysema Treatment
The purpose of this study is to confirm the efficacy and safety of BaofeiKang Granule in the treatment of Combined Pulmonary Fibrosis and Emphysema patients.
45 Years - 75 YearsLearn More -
A Longitudinal Study of Hermansky-Pudlak Syndrome Pulmonary Fibrosis
Hermansky-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...
12 Years - 90 YearsLearn More -
American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis
Vasculitis is group of diseases where inflammation of blood vessels is the common feature. Patients typically present with fever, fatigue, weakness and muscle and joint aches. These symptoms are very common among many different diseases, not just vasculitis. A clustering of other symptoms, physical examination findings, blood tests, radiology and biopsy help make the diagnosis. There are currently no criteria to help doctors make a diagnosis of vasculitis when a patient presents with these non specific symptoms and they are reliant on previous experience and disease definitions. One of the aims of this project is to develop diagnostic ...
18 Years and OverLearn More -
A Pilot Trial of Herpesvirus Treatment in Idiopathic Pulmonary Fibrosis (IPF)
The investigators will conduct a single-center, prospective, randomized, placebo-controlled, double-blind pilot study of anti-herpesvirus therapy in patients with idiopathic pulmonary fibrosis (IPF). Patients with mild, moderate or severe IPF with serologic evidence of current or past Epstein-Barr Virus (EBV) or cytomegalovirus (CMV) infection. Randomization will be to pirfenidone plus placebo or pirfenidone plus valganciclovir. Thirty subjects will be enrolled and randomized to treatment with pirfenidone plus valganciclovir (20 subjects) or pirfenidone plus placebo (10 subjects) for 12 weeks. The primary outcome will be safety and tolerability ...
21 Years - 80 YearsLearn More -
APRIL (AbatacePt in RA-ILD)
Early initiation of treatment for Rheumatoid arthritis (RA) can prevent several of the long term problems associated with the condition. However, many RA patients develop lung inflammation and scarring, called 'interstitial lung disease' (RA-ILD), contributing to early death in 1 in 5 people. There is no proven treatment for these patients and some medications for RA can in fact worsen their lung disease. There is an urgent need therefore to find safe medications that can not only control RA joint disease, but also prevent progression of RA-ILD. Abatacept is an approved drug for treating RA and is used widely. It is a newer RA medication,...
18 Years and OverLearn More -
Assessment of Lung Movement With Computed Tomography (CT)
The purpose of this study is to determine whether patient with ILD have altered lung compliance on chest CT before they have macroscopic structural changes on CT.
18 Years and OverLearn More -
Assessment of Peripheral Endothelial Function in Idiopathic Pulmonary Fibrosis
The aim of the study is to assess the peripheral endothelial function in adult patients with idiopathic pulmonary fibrosis (IPF) and the relationship between the peripheral endothelial function and the severity of the IPF.
18 Years - 85 YearsLearn More -
Assessment of Safety of Air Travel in Patients With Pulmonary Langerhans Cell Histiocytosis
The aim of this study is to conduct survey-based assessments for the safety of air travel in patients with pulmonary Langerhans cell histiocytosis (PLCH). The study will enroll patients through the clinic network at Rare Lung Disease Consortium (RLDC) and through the Histiocytosis Association website. Patients will have access to the questionnaire via REDCap (an online data management system) and each patient will be provided with a link to complete the survey. The investigators plan on enrolling approximately 200 patients with PLCH for the purpose of this study. Secondary aims of this study include further characterization of the clinical...
18 Years and OverLearn More
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