Active clinical trials for "Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis"

ANCA vasculitis is a pauci-immune systemic small vessel vasculitis. The anti-neutrophilic cytoplasmic antibodies (ANCA) are pathogenic and cause disease by activating neutrophils which damage blood vessels. CD means "cluster of differentiation" . CD5 is a type I transmembrane protein found on T cells, thymocytes, and some B cells. CD20 is a type III transmembrane protein found on B cells. The investigators previously detected an association between recovery of Interleukin 10 (IL-10)-secreting CD20+ and CD5+ regulatory B cells after immunotherapy (with rituximab and corticosteroids) and decreased risk of subsequent relapse in patients with ANCA-vasculitis. The investigators hypothesize that patients with complete reconstitution of a functional regulatory B cell repertoire after induction therapy are at low risk of relapse and may be monitored conservatively without further immunotherapy. The investigators will test this hypothesis through a proof of concept randomized controlled study. Patients with normalization of CD5+ regulatory B cells will be randomized to maintenance therapy with rituximab vs. close observation without immunosuppression. Patients whose peripheral CD5+ regulatory B cells remain low after induction therapy (who are at higher risk of relapse), will receive maintenance immunosuppression with rituximab. Patients needing or randomized to maintenance therapy who are unable to receive rituximab will receive azathioprine or mycophenolate mofetil, two standard alternative medications for maintenance immunosuppression.

The 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).

This prospective randomized trial aims to evaluate the feasibility, risk and benefit of the discontinuation of immunosuppressive maintenance treatments in AAV (Antineutrophil Cytoplasmic Autoantibodies (ANCA)-associated vasculitis) patients who have reached ESRD (end-stage renal disease). Our hypothesis is that discontinuation of immunosuppressive therapy in AAV patients with ESRD will not expose these patients to an excessive risk of extra-renal AAV relapse, while reducing the rate of complications due to immunosuppression, particularly infections. Patients with ESRD related to AAV will be randomized into 2 arms: arm 1: discontinuation (or not initiation) of maintenance treatment (Experimental group) arm 2: maintenance (or initiation) of immunosuppressive treatment (Control group). The main objective of this study is to demonstrate a superiority of immunosuppression discontinuation in ESRD-AAV patients compared to standard maintenance immunosuppressive therapy in terms of severe prejudicial event-free survival at 24 months. The second objectives include the frequency of major and minor relapses, of infectious episodes and leukopenia in both groups and the establishment of a prospective database regarding the outcome of ESRD-AAV patients.

As rare disease, vasculitis affects a small number of patients, the cohorts available in the literature are few and the pathophysiological mechanisms remain to be elucidated. The collection of standardized data within a patientheque as part of a multi-year follow-up will facilitate the study of the characteristics of these diseases. This may, in particular, address the main objective of identifying predictors of relapse, as well as secondary objectives for predictive factors of mortality, infectious, cardiovascular or neoplastic complications that affect the prognosis of vasculitis in order to establish a more appropriate management of the patients concerned.

The study aims at defining the role of soluble CD95 Ligand in the physiopathology of a rare group of inflammatory diseases: ANCA associated vasculitis. Soluble CD95 Ligand might have a prognostic and diagnostic interest as well as potential for the discovery of new therapeutic strategies.

Mechanistic study to assess whether dual B-cell immunotherapy by co-administration of rituximab and belimumab will result in improvements in biological endpoints, functional outcomes and clinical status compared to rituximab with placebo.

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 will have the option of entering an open-label trial period whereby they would receive open-label abatacept.

Background: - Vasculitis is a group of diseases that inflame and damage blood vessels and tissue. It can cause many medical problems. Few tests can diagnose the disease, and none can reliably predict a relapse. Researchers want to study people s genes and follow people over time to see how the disease affects them. Objective: - To learn the signs, symptoms, imaging tests, genetic markers, and blood tests that can help identify people with vasculitis and predict what will happen to them over time. Eligibility: People age 3 and older who have or are thought to have vasculitis, or are related to someone with it. Healthy volunteers. Design: Participants will be evaluated by a doctor who has expertise caring for patients with vasculitis. Participants will give a blood sample. Some will give a urine sample. Some participants may have brushings or biopsies taken from the inside lining of the nose. Images of participants blood vessels may be taken using scans. For some scans, participants will lie on a table that moves in and out of a cylinder that takes pictures. For some scans, a contrast agent may be injected into an arm vein. Other scans may use a radioactive form of sugar. Healthy minors will not have scans. Some participants will answer questionnaires. - Some participants will have their tests done at NIH. Others will have their doctor take the blood, saliva, or cheek swab samples and send them to NIH. Some participants will have one visit lasting 1-2 (but sometimes up to 4) days. Some participants may have follow-up visits every 3 - 6 months, indefinitely.

This study aimed to explore the incidence of Anti-neutrophil cytoplasmic antibody (ANCA）-associated vasculitis (AAV) progression and its association with adverse consequences. It will enroll approximately 500 AAV patients in Hunan province of China and follow-up for at least 5 years. Demographic characteristics, clinical and laboratory data will be collected at baseline and every follow-up. The principal clinical outcomes of the study consist of end stage renal disease (ESRD) and death.

This is a prospective observational study to determine the frequency and magnitude of Cytomegalovirus (CMV) reactivation in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) in the acute phase of the disease (within 12 months of diagnosis or relapse and commencement of induction of remission therapy) and its association with clinical outcomes. The investigators will also explore whether CMV reactivation causes an increase in CCR2 expressing monocytes, and whether these monocytes cause persistent kidney damage in AAV. The investigators hypothesise that reactivation of CMV during the initial 12 months following diagnosis or relapse of AAV occurs frequently but is generally asymptomatic. Based on the investigators' preliminary data the investigators further hypothesise that subclinical reactivation of CMV during this period will be associated with adverse clinical outcomes, including the severity of vasculitis, the response to treatment and the damage caused by vasculitis. Finally, they hypothesise that subclinical CMV reactivation leads to amplification of renal damage in AAV through a monocyte CCR2/CCL2 driven pathway. The investigators' research has recently shown that asymptomatic reactivation of CMV is a frequent event in AAV patients, occurring in roughly 25% of AAV patients in remission. However, the frequency of asymptomatic reactivation of CMV during the acute phase of the disease is not known. The investigators have previously shown that CMV infection and surrogate markers of CMV reactivation in patients with AAV are associated with worse outcomes such as reduced kidney function, increased risk of infection and death, increased risk of blood clots and increased stiffness of the blood vessels, which is a risk factor for heart disease and stroke. The investigators also have preliminary findings suggesting that in patients with AAV and CMV reactivation, the more CCR2 expressing monocytes in the blood, the worse the kidney function. If CMV reactivation during the acute phase of the disease is common and linked with worse outcomes, this study may then lead on to future research involving treatment to prevent CMV reactivation aiming to improve patient outcomes. The investigators will be looking to recruit patients under the care of the Queen Elizabeth Hospital with newly diagnosed or recently relapsed AAV in the last 2 weeks who are positive for previous CMV infection.The investigators will follow these patients up with 10 visits over 12 months; where possible these will coincide with participants' usual vasculitis clinic appointments. At each visit the participants will be required to give blood and urine samples and answer questions related to their vasculitis. Kidney biopsy tissue taken at diagnosis will be used to assess mechanisms of injury during CMV reactivation.