Reslizumab in the Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA) Study
AsthmaReslizumab is a type of medicine called a monoclonal antibody that is made in the research clinic; it works by blocking a specific protein in the body called interleukin-5. The study medicine, reslizumab, is not yet approved for doctors to treat patients with EGPA. It is considered an experimental drug in this study.
CHUSPAN SCS BP Treatment of Churg-Strauss Syndrome Without Poor-Prognosis Factors
Churg-Strauss SyndromeTo assess the efficacy of systemic corticosteroids alone as first-line treatment of Churg-Strauss syndrome without poor-prognosis factors as defined by the five-factor score (FFS=0), and to compare the efficacy and safety of azathioprine vs pulse cyclophosphamide as adjunctive immunosuppressive therapy to treat failure or relapse
Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune...
PurpuraSchoenlein-Henoch15 moreOBJECTIVES: I. Determine whether there is prompt engraftment after autologous peripheral blood stem cell transplantation using filgrastim (G-CSF) mobilization in patients with life threatening autoimmune diseases. II. Determine the kinetics of T- and B-cell immune reconstitution after a combination of timed plasmapheresis, high dose cyclophosphamide and total lymphoid irradiation, and posttransplant immunosuppression with cyclosporine in these patients. III. Determine whether this treatment regimen beneficially influences the clinical course of these patients.
Eosinophilic Granulomatosis With Polyangiitis Cohort
Churg-Strauss SyndromeEGPA (Eosinophilic Granulomatosis with Polyangiitis, previously Churg-Strauss syndrome) is a rare vasculitis, characterised by asthma, rhinosinusitis and eosinophilia. There has previously been confusion around diagnostic criteria, with multiple sets of classification criteria being used inappropriately for diagnosis. The ERS formed a taskforce to produce specific diagnostic criteria, and these divided the Churg-Strauss Syndrome cohort into two groups - vasculitic EGPA and tissular Hypereosinophilic Asthma with Systemic Manifestations (HASM). It is not known if the groups separated by the diagnostic criteria are also separated in clinical symptomatology, or if their disease course varies, and this could have significant impact on management and further research.
The ANCA Vasculitis Questionnaire (AAV-PRO©)
Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) (EGPA)Churg-Strauss Syndrome (CSS)5 moreThe 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).
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).
VCRC Patient Contact Registry Patient-Reported Data Validation Study
Behcet's DiseaseChurg-Strauss Syndrome5 moreThe purpose of this study is to provide validation of patient-reported data in the VCRC Patient Contact Registry by comparing patient-reported data with data provided by the physician who is the primary provider caring for the patient's vasculitis. Patients enrolled in the Patient Contact Registry with Behcet's disease, eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), giant cell arteritis (GCA), granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK) were invited via email to participate in this study.
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
Journey of Patients With Vasculitis From First Symptom to Diagnosis
VasculitisSystemic Vasculitis14 moreThis study seeks to understand the journey that patients eventually are diagnosed with vasculitis experience in the period prior to their formal diagnosis by a healthcare provider. Data elements of interest include average time from the onset of the first symptoms to the time a diagnosis of vasculitis is confirmed. Other aims include identifying factors associated with the time to diagnosis. These factors will be divided into: a) intrinsic factors, or so-called "patient-related factors", such as the type of vasculitis symptoms, patient demographics, socioeconomic status, patients' beliefs regarding the etiology of their symptoms, and other factors, and b) extrinsic factors, or "professional/health system factors", such as healthcare access, referral patterns, testing patterns, and other factors. Understanding such factors can guide future efforts to shorten delays in diagnosis and thereby improve outcomes. All analyses will be done for the population of patients with vasculitis as a whole and by individual types of vasculitis.
Reproductive Health in Men and Women With Vasculitis
Giant Cell ArteritisTakayasu's Arteritis10 moreThe purpose of this study is to learn about reproductive health, including fertility and pregnancies, in people with vasculitis.