Defining Immune Tolerance in ANCA-associated Vasculitis (AAV)
ANCA-Associated VasculitisThe 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.
CMV Modulation of the Immune System in ANCA-associated Vasculitis
ANCA Associated VasculitisCMV InfectionThe 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.
WEGENT - Comparison of Methotrexate or Azathioprine as Maintenance Therapy for ANCA-Associated Vasculitides...
Systemic Wegener's GranulomatosisRemission 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.
Spontaneous Coronary Artery Dissection (SCAD) and Autoimmunity
SCADAddison Disease28 moreThis case control study aims to determine whether spontaneous coronary artery dissection (SCAD) is associated with autoimmune diseases and to update the incidence of SCAD in a population-based cohort.
Giant Cell Arteritis and Differential Diagnoses Associated With Positive Temporal Artery Biopsy...
Giant Cell ArteritisVasculitisTemporal artery biopsy is a useful tool helping to the diagnosis of giant cell arteritis. However, other diagnoses might be associated with abnormal temporal artery biopsy. The purpose of this study is to describe the frequency of giant cell arteritis differential diagnoses with positive temporal artery biopsy.
Educational Needs of Patients With Systemic Vasculitis
Behcet's DiseaseChurg-Strauss Syndrome8 moreA cross-sectional study design and online questionnaire was used to assess the informational needs of patients with several different types of systemic vasculitis. Patients were recruited from within the Vasculitis Clinical Research Consortium (VCRC) online Patient Contact Registry1. Survey responses from participants in the VCRC Patient Contact Registry were compared to responses from a similar survey recently administered to patients within a United Kingdom (UK) based vasculitis support group (Vasculitis UK).
TransIent Perivascular Inflammation of the Carotid Artery (TIPIC) Syndrome: Clinical and Radiological...
Cervical PainVasculitisTIPIC syndrome (previously called carotidynia) is a rare entity of unknown cause, where the patient has a temporary cervical pain associated with a suggestive inflammation around the carotid artery. Usually considered as a unique and benign episode, some patients have recurrences and the follow-up is usually short. In this study the investigators aim to evaluate patients who presented a TIPIC episode at least 3 months before, clinically and with a cervical doppler ultrasound
MICRO-RNAs OF NEUTROPHILS IN RENAL ANTINEUTROPHIL CYTOPLASMIS ANTIBODY (ANCA) -ASSOCIATED VASCULITIS...
ANCA-associated VasculitisActivation 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.
Impact of Vasculitis on Employment and Income
VasculitisSystemic Vasculitis11 moreThe purpose of this study is to learn about the impact of vasculitis on employment and income in patients with different systemic vasculitides. All patients enrolled in the Vasculitis Clinical Research Consortium (VCRC) Patient Contact Registry, living in USA or Canada, and followed for more than 1 year since the vasculitis diagnosis will be invited via email to participate in this study, based on an online survey.
Vasculitis Illness Perception (VIP) Study
Wegener GranulomatosisMicroscopic Polyangiitis7 moreThe purpose of this study is to learn about how patients with vasculitis think about their illness and to assess to what extent patient perceptions of illness are associated with physical, mental, and social functioning