Hydroxychloroquine in ANCA Vasculitis Evaluation
ANCA Associated VasculitisMicroscopic Polyangiitis2 moreThe purpose of this study is to find out whether hydroxychloroquine, in addition to background treatments, reduces disease activity in patients with Anti-Neutrophilic Cytoplasmic Autoantibodies (ANCA) Vasculitis, a group of autoimmune diseases. Hydroxychloroquine and is an established, effective, safe and inexpensive therapy, widely used in other autoimmune diseases such as lupus and rheumatoid arthritis. The study is open to adults diagnosed with certain types of vasculitis, called Granulomatosis Polyangiitis (GPA), Microscopic Polyangiitis (MPA) or Eosinophilic Granulomatosis with Polyangiitis (EGPA). Participants will be eligible if they are treated with background medication to control their vasculitis disease and have a low level of disease activity as defined by a Birmingham Vasculitis Activity Score (BVAS) of greater than 3. Participants will be randomly placed in 1 of 2 groups. Both groups will be given background medication. One group will receive hydroxychloroquine and the other will receive placebo. Participants will be on treatment for 1 year. 76 ANCA Vasculitis participants will be recruited (38 in each treatment arm) from UK vasculitis specialist centres.
Low Dose Naltrexone to Improve Physical Health in Patients With Vasculitis
Eosinophilic Granulomatosis With Polyangiitis (EGPA)Churg-Strauss Syndrome (CSS)5 moreNaltrexone is an FDA approved drug (for alcoholism) that has found widespread use "off-label" to treat pain and improve quality of life at much lower doses than are used for the approved indication. There are a few scientific studies in three conditions (fibromyalgia, Crohn's disease, and multiple sclerosis) that suggest that this drug has benefit and is safe. However, considering the extent of use in other conditions, and uncertainty about the mechanism of action study is needed in a diverse set of diseases, including vasculitis. The purpose of this clinical trial is to determine if low dose naltrexone is effective in improving health-related quality of life (HRQoL) among patients with vasculitis. Although it is a pilot study, a placebo-controlled component is used because of the prominent placebo group effect seen in studies with self-reported subjective outcomes.
PR3-AAV Resilient Remission or PRRR
Granulomatosis With PolyangiitisMicroscopic Polyangiitis1 moreThe purpose of this study is to evaluate the efficacy and safety of obinutuzumab for the treatment of proteinase 3 Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (PR3-AAV).
Efficacy and Safety of Rituximab in the Treatment of Good Prognosis Microscopic Polyangiitis
Microscopic Polyangiitis (MPA)The purpose of the study is to determine wether a rituximab-based treatment compared to standard therapy (glucocorticoid alone) in patients with microscopic polyangiitis without any bad prognosis marker increases the remission and reduces the relapse free survival rate.
Pediatric Vasculitis Initiative
Wegeners Granulomatosis (Granulomatosis With Polyangiitis)Microscopic Polyangiitis5 moreChildhood chronic vasculitis describes a group of rare life-threatening diseases that have in common inflammation of blood vessels in vital organs such as kidneys, lungs and brain. Most knowledge about them comes from adult patients. Severe disease requires aggressive life-saving treatments with steroids and some cancer drugs which can themselves cause damage, and increase risks of cancer and severe infections. Conversely, milder disease can be treated with less toxic drugs. Different classification and "scoring tools" are used to define the types and severity of vasculitis and to measure damage caused by disease or drugs. These in turn help direct how aggressively to treat a patient and to measure outcome. None of these tools however have been assessed in children and the best balance of disease and treatment risks against outcome for children is not known. Although causes of these diseases in children and adults are probably the same, the effects of the disease and the response (good and bad) to drugs will differ in growing children. Because specialists may see only one new child with vasculitis each year, obtaining enough information to learn about childhood vasculitis requires cooperation. We will use an international web-based registry to which doctors from 50 or more centers can contribute patient data. We will determine the features which help better classify and diagnose children compared to adults. Through the web we will collect and analyze information on patients similarly classified and "scored" so that most successful treatments can be identified. Children with vasculitis are less likely to have diseases associated with aging, alcohol and smoking etc., and therefore may be a better group in whom to study the underlying biology of vasculitis. We will use this opportunity and collect spit, blood and tissue from registry patients for laboratory study with an aim to find biomarkers to better classify, define and direct optimal treatment and outcomes.
Establishment of an ELISA for the Recognition of Procalcitonin Variants in Patients With Hyperprocalcitonemia....
SepsisPre-Eclampsia5 moreProcalcitonin is a protein consisting of 116 amino-acids which can rapidly rise under inflammatory conditions and sepsis. More than 20 years ago it has been shown that dipeptidylpeptidase-4 (DPP-4) cleaves procalcitonin from the n-terminus, resulting in a truncated procalcitonin-variant which consists of 114 aminoacids. Within our workgroup we found that the truncated procalcitonin-variant had deleterious effects on vascular integrity during sepsis in mice. However, it is unknown if this applies also in humans. By using an ELISA-assay we want to examine the ratio between native and truncated human procalcitonin during diseases accompanied with hyperprocalcitoninemia and correlate the results with clinical data.
One-Time DNA Study for Vasculitis
Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss)Giant Cell Arteritis4 moreThe purpose of this study is to identify genes that increase the risk of developing vasculitis, a group of severe diseases that feature inflammation of blood vessels. Results of these studies will provide vasculitis researchers with insight into the causes of these diseases and generate new ideas for diagnostic tests and therapies, and will be of great interest to the larger communities of researchers investigating vasculitis and other autoimmune, inflammatory, and vascular diseases.
Biosimilars of Rituximab in ANCA-associated Vasculitis Compared to the Originator
ANCA-associated VasculitisGranulomatosis With Polyangiitis1 moreThe goal of this multicentre observational study is to compare the safety and effectiveness of rituximab biosimilars to the originator in Canadian patients with Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA), two main forms of ANCA-associated vasculitis (AAV). The main questions it aims to answer are: Is there a difference in vasculitis control between originator and biosimilar rituximab? Is there a difference in adverse effects between originator and biosimilar rituximab? In the Canadian healthcare context, are wait times to receive approval (financial coverage) for rituximab shorter for biosimilars compared to originators? Investigators will perform study assessments (including recording disease activity, damage, and adverse events) at the time of participants' usual clinical care visits, at regular intervals for 2 years after starting rituximab (for induction or maintenance treatment) or switching from an originator to a biosimilar as part of their usual care. Researchers will compare outcomes among participants who have received rituximab originators (from 2018 onwards) or biosimilars as part of their usual care, to see if there are differences in relapses, remission rates, damage, serious infections, serious adverse events, and treatment approval wait times.
Vasculitis Pregnancy Registry
VasculitisBehcet's Disease13 moreThe purpose of this study is to learn about the experience of women with vasculitis who become pregnant. In particular, the study will consist of several online surveys to assess each woman's vasculitis severity and pregnancy-related experiences, and pregnancy outcomes.
VCRC Tissue Repository
AortitisCutaneous Vasculitis9 moreThe purpose of this study is to collect existing tissue specimens from subjects enrolled in Vasculitis Clinical Research Consortium (VCRC) studies. Analysis of these tissue specimens and linked clinical data collected through VCRC studies may lead to the identification and development of a series of translational research projects. Results of these studies will provide vasculitis researchers with insight into the causes of these diseases and generate new ideas for diagnostic tests and therapies, and will be of great interest to the larger communities of researchers investigating vasculitis and other autoimmune, inflammatory, and vascular diseases.