Comparison of ANCA and Anti-GBM Auto-antibodies Removal Kinetics Between Plasma Exchanges and Immunoadsorption...
Kidney FailureAcuteAnti-neutrophil cytoplasmic antibodies (ANCA), directed against myeloperoxidase (MPO) and against proteinase 3 (PR3), have a pathogenic role during ANCA (AAV) vasculitis. Glomerular basement membrane (MBG) antibodies also have a direct pathogenic role in Goodpasture's syndrome and anti-MBG antibody glomerulonephritis (GN). In some patients, the severity of renal and / or pulmonary involvement justifies the rapid purification of these autoantibodies by an apheresis procedure, while waiting for the effect of immunosuppressive treatments aimed at reducing their production. During vasculitis, plasma exchange (PE) is recommended in patients with severe renal impairment or intra-alveolar hemorrhage (2012 KDIGO Clinical Practice Guideline for Glomerulonephritis). Given certain disadvantages related to plasma exchanges (low volume of purified plasma, non-selective technique for immunoglobulins (Ig), need for replacement solute, induction of coagulation disorders), immunoadsorption (IA), already used in transplantation, has been developed in these indications. IA has indeed greater selectivity for Ig with a probable better purification capacity due to higher volumes of plasma treated per session. The price of IA is however higher than that of EP. These two apheresis techniques, EP and IA, are commonly used in France during severe forms of vasculitis ANCA or anti-MBG, without the superiority of one or the other has been demonstrated. As a result of higher plasma volumes being purified, AI may allow faster purification of pathogenic antibodies. No studies to date have specifically compared the purification kinetics of these antibodies between EP and IA. The CINEVAS study (VAScularite Antibody Purification CINetic) is a multicentric pilot study whose main objective is to compare the purification kinetics of ANCA (anti-MPO or anti-PR3) and / or anti- MBG in patients treated with EP versus those treated with IA
Exploring Durable Remission With Rituximab in Antineutrophil Cytoplasmic Antibody(ANCA)-Associated...
ANCA Associated VasculitisMost recent insights in the treatment for patients with ANCA-associated vasculitis (AAV) have demonstrated that 'tailored' maintenance treatment with rituximab (RTX) is effective to achieve durable remission of disease. As such, RTX re-treatment can be tailored on the basis of relevant clinical and immunological parameters in AAV patients. Now, the present study intends to evaluate whether combining rituximab with cyclophosphamide is superior to current standard of care with rituximab only to induce a favorable clinical and immunological state in AAV patients and can thereby reduce the number of tailored re-treatments with rituximab.
Comparison of the Efficacy of Leflunomide and Azathioprine for the Maintenance Therapy of ANCA Associated...
ANCA Associated VasculitisMaintenance TherapyThis study is a prospective, open-labelled, randomized, controlled,multi-center clincial trial. The aim of this study is to verify that the remission rate of patients treated with Leflunomide is not inferior to that of patients treated with Azathioprine.
Autologous Stem Cell Transplant for Neurologic Autoimmune Diseases
Autoimmune DiseaseNeurologic Autoimmune Disease17 moreThis phase II trial studies the side effects and how well carmustine, etoposide, cytarabine and melphalan together with antithymocyte globulin before a stem cell transplant works in treating patients with autoimmune neurologic disease that did not respond to previous therapy. In autoimmune neurological diseases, the patient's own immune system 'attacks' the nervous system which might include the brain/spinal cord and/or the peripheral nerves. Giving high-dose chemotherapy, including carmustine, etoposide, cytarabine, melphalan, and antithymocyte globulin, before a stem cell transplant weakens the immune system and may help stop the immune system from 'attacking' a patient's nervous system. When the patient's own (autologous) stem cells are infused into the patient they help the bone marrow make red blood cells, white blood cells, and platelets so the blood counts can improve.
A Randomized Multicenter Study for Isolated Skin Vasculitis
Primary Cutaneous VasculitisCutaneous Polyarteritis Nodosa2 moreMulti-center sequential multiple assignment randomized trial comparing the effectiveness of three different standard of care treatment options for patients with isolated skin vasculitis.
ETA and AT1 Antagonism in ANCA-vasculitis (SPARVASC)
ANCA Associated VasculitisCardiovascular Diseases1 moreANCA-associated vasculitis is an autoimmune disease that causes damage to blood vessels. This leads to organ damage with the number of organs affected and the severity of damage varying significantly between patients. Vasculitis patients also have a very high risk of heart attacks and strokes, called cardiovascular disease. A chemical called 'endothelin', produced by the blood vessels, causes vessels to stiffen and raises blood pressure and this associates with cardiovascular risk. The investigators have previously shown that by blocking the effects of endothelin you reduce vessel stiffness, lower blood pressure and improve vessel function. However, these studies only blocked endothelin for a few hours. Now, the investigators would like to see if it is possible to maintain these benefits by blocking endothelin for longer. Sparsentan is a tablet that blocks endothelin and lowers blood pressure. The investigators plan to give sparsentan to patients with vasculitis for 6 weeks. To determine if any beneficial effects of sparsentan are due to blood pressure lowering the investigators will give another group of vasculitis patients a tablet called irbesartan which lowers blood pressure but does not block endothelin. The investigators will compare the results between the two groups.
Efficacy and Safety of Substitution of Glucocorticoid for BDB-001 Injection in Patients With Anti-neutrophil...
ANCA-associated VasculitisThe aim of the trial is to study the efficacy and safety of treatment with BDB-001 Injection substitution of glucocorticoid in patients with ANCA-associated vasculitis.
Safety and Efficacy of CD19 Targeted CAR-T Therapy for Refractory Autoimmune Disease
SLE (Systemic Lupus)Sjogren's Syndrome3 moreThis is a single arm study to evaluate the efficacy and safety of CD19 targeted CAR-T cells therapy for patients with Refractory Autoimmune Disease
A Clinical Study of CD19/BCMA CAR-T Cells in the Treatment of Refractory POEMS Syndrome, Amyloidosis,...
POEMS SyndromeAmyloidosis2 moreA Clinical Study on the Safety and Effectiveness of CD19/BCMA Chimeric Antigen Receptor T Cells in the Treatment of Refractory POEMS Syndrome, Amyloidosis, Autoimmune Hemolytic Anemia, and Vasculitis
Efficacy and Safety for Rituximab Combined With Telitacicept in the Treatment of ANCA-associated...
ANCA Associated VasculitisThis study is a prospective, open-labelled, randomized, controlled, single-center clinical trial. The aim of this study is to investigate the remission rate of patients treated with Telitacicept combined with Rituximab in remission-induction and Telitacicept alone in remission-maintain treatment.