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

Previous reports suggested conventional immunosuppressants such as cyclophosphamide could not reduce glucocorticoid dose in remission induction in ANCA-associated vasculitis because of lower remission rate and higher relapse rate. However those reports didn't include rituximab. B cell depletion therapy by rituximab is a new strategy for remission induction in ANCA-associated vasculitis. The RAVE and RITUXVAS trial (NEJM 2010, both) showed high-dose glucocorticoid plus rituximab had roughly the same efficacy and safety as high-dose glucocorticoid plus IV-cyclophosphamide. In addition, recent retrospective observational studies reported low-dose glucocorticoid plus rituximab led to re-induction in severe relapsing ANCA-associated vasculitis. Thus, the investigators aim to investigate whether rituximab can reduce glucocorticoid dose in induction remission in ANCA-associated vasculitis (to show non-inferiority for efficacy between low-dose and high-dose glucocorticoid plus rituximab). Participants will be randomised to the "low-dose glucocorticoid plus rituximab" or the high-dose glucocorticoid plus rituximab" groups. Primary endpoint is proportion of remission at 6 months, then data regarding relapse and long-term safety will be collected until 24 months. The study has been designed by the principal and coordinating investigators. It will include 140 participants from 18 hospitals in Japan. It is funded by Chiba University Hospital and Chiba East Hospital.

The aim of the investigators' study is to evaluate whether monitoring serum rituximab levels could be an interesting tool in the follow-up of ANCA-associated vasculitis patients. All consecutive patients, hospitalized for a new diagnosis of ANCA-associated vasculitis or the relapse of a known ANCA-associated vasculitis, in which the decision to start an induction regimen with rituximab has been taken, will be included. Serum rituximab levels (along with serum anti-rituximab antibodies levels) will be determined (at M+1 and M+3) and the correlation with clinical outcome at M+6 will be analyzed.

The aim of this project is to develop a disease specific patient reported outcome measure (PROM) for patients with AAV (the AAV-PRO). Investigators are developing and validating a questionnaire to assess quality of life in patients with ANCA-associated vasculitis (AAV). Patients with AAV have inflammation in the small blood vessels leading to involvement of a range of organs and can suffer from ongoing disease activity or treatment side effects. Quality of life can be measured by patient reported outcome measures (PROMs).

The goal of the study is to find biological markers (certain proteins or cellular markers found in a blood test) that will inform doctors which patients diagnosed with ANCA-associated vasculitis (AAV) are most likely to be able to stop their medications suppressing their immune systems and remain in remission.

The purpose of this study is to determine whether Cytomegalovirus (CMV) reactivation in ANCA-associated vasculitis (AAV) patients can be effectively and safely reduced using an antiviral agent (valaciclovir) and whether this in turn improves the function of the immune system thereby also improving the body's ability to fight other infections. The primary hypothesis is that repeated episodes of CMV reactivation in AAV patients drive the expansion and functional impairment of CMV-specific T-cells, with increased susceptibility to infection. Inhibition of CMV replication with valaciclovir will block further stimulation of CMV specific T-cells and increase the functional capacity of the immune system.

Remission of ANCA-associated vasculitis can be obtained in approximately 80% of the patients with a combination of corticosteroids and cyclophosphamide. However, relapses are frequent. This point warrants the prescription of a maintenance treatment with a less toxic immunosuppressant for several months to years. The optimal drug in this indication is not determine. We decided therefore to compare the 2 most used drugs in this indication. Induction therapy consists in the combination of corticosteroids and intravenous cyclophosphamide pulses. Corticotherapy consisted first in one daily methylprednisolone pulse, for 1 to 3 days, followed by oral prednisolone at the dose of 1 mg/kg/d for 3 weeks, then progressively tapered and stopped at the 18th month from the diagnosis. Cyclophosphamide is administered every 2 weeks for the first 3 bolus (0.6 g/m2 - D1, 15 and 30), then every 3 weeks (0.7 g/m2). Once remission is achieved, patients receive 3 additional bolus (0.7 g/m2). At that time, patients are randomized for a maintenance treatment with azathioprine (2 mg/kg/d, orally) or oral methotrexate (starting at the dose of 0.3 mg/kg/wk, then progressively increased every weeks by 2.5mg, if necessary, to a maximum and optimal dose of 25 mg/wk) for 12 months.

Activation of neutrophils by ANCA ("Anti-Neutrophil Cytoplasm Antibodies") and subsequent microvascular endothelial cell damage is the main feature of ANCA-associated vasculitis (AAV), a severe autoimmune disease that often targets the kidney. There is no specific treatment for AAV to date and 25% of patients with renal damage evolve towards end-stage renal disease, requiring dialysis and kidney transplantation. In addition, there is no reliable biological marker of the disease activity available, which makes the diagnostic, follow-up and treatment of patients difficult. Therefore, the identification of new therapeutic targets and non-invasive biomarkers constitutes a major clinical challenge to improve AAV patients care and to ameliorate their renal outcome.

Descriptive study of the residual anti-pneumococcal immunity in patients with Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) who have previously gone through pneumococcal immunization.

The purpose of this study is to investigate the clinical features and long-term outcome of anti-neutrophil cytoplasmic antibody (ANCA)-positive interstitial lung disease (ILD) and assess the difference between microscopic polyangiitis (MPA) associated ILD and isolated ANCA-positive idiopathic interstitial pneumonia.

ANCA vasculitis and Sjögren syndrome are two rare diseases, and even more rarely associated. These two conditions have specific organ involvements, and specific follow-up. The Investigators hypothesise that patients with co-occurrence of these two diseases may have a singular clinical course.