Biocollection of Patients With ANCA Associated Vasculitis Diagnosed Within the CERAINO Autoimmune Disease Reference Center, Part of the Global BRAISE Project (B-dependent Rare AutoImmune DiseaSES
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 100
- 试验地点
- 2
- 主要终点
- Relapse-free survival of the disease
研究概览
简要总结
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.
详细描述
Vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA) is a group of rare and severe autoimmune diseases, encompassing several entities: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (PMA), and eosinophilic granulomatosis with polyangiitis (GEPA). When untreated, these diseases are fatal in a matter of months. Currently, thanks to the use of corticosteroids and immunosuppressants, this high mortality has greatly decreased and these are now chronic diseases. On the other hand, these patients are at high risk of morbidity, linked to both relapses (occurring in at least 50% of patients) and side effects of treatments. It is therefore essential to be able to define which patients are at risk of relapse and justify long-term immunosuppressive treatment to avoid recurrence of the disease, and conversely which patients have a low risk of relapse and in whom immunosuppressive treatments can be discontinued to limit the risk of side effects. However, so far no predictor or biomarker can accurately assess this risk of relapse.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Diagnostic
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 99 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •Major patients with no upper age limit.
- •Patients assessed as part of the reference centre for rare autoimmune diseases at the CHRU in Brest.
- •Patients for whom a diagnosis of ANCA-associated vasculitis is made by the physician in charge of the patient, according to the definitions of the Chapel-Hill Consensus Conference.
- •Patient affiliated with Social Security
- •Patient who has signed written informed consent
排除标准
- •Patients unable to consent.
- •Patients refusing to participate in research
- •Patient under legal protection (tutelage, curatorship)
- •Pregnant or lactating women
- •Hemoglobin (Hb) < 7g/dL
研究组 & 干预措施
ANCA-associated vasculitis - patient library
It is a description of ANCA-associated vasculitis patients cohort. All the patients are included in one arm. They will undergo various type of samples.
干预措施: Blood samples (80 mL) (Other)
ANCA-associated vasculitis - patient library
It is a description of ANCA-associated vasculitis patients cohort. All the patients are included in one arm. They will undergo various type of samples.
干预措施: Fecal samples (Other)
ANCA-associated vasculitis - patient library
It is a description of ANCA-associated vasculitis patients cohort. All the patients are included in one arm. They will undergo various type of samples.
干预措施: Urinary sample (20-40 mL) (Other)
ANCA-associated vasculitis - patient library
It is a description of ANCA-associated vasculitis patients cohort. All the patients are included in one arm. They will undergo various type of samples.
干预措施: Questionnaires (Other)
结局指标
主要结局
Relapse-free survival of the disease
时间窗: Five years after diagnosis
Relapse-free survival of the disease
次要结局
- Patient assessment of disease activity(Five years after diagnosis)
- VDI score - Vasculitis Damage Index(Five years after diagnosis)
- Age(Five years after diagnosis)
- Sex(Five years after diagnosis)
- HAQ-DI - Health Assessment Questionnaire - Disability Index.(Five years after diagnosis)
- Number of patients with refractory character of the Vasculitis(Five years after diagnosis)
- Glucocorticoid toxicity index - Glucocorticoid toxicity index during follow-up Glucocorticoid toxicity index(Five years after diagnosis)
- death(Five years after diagnosis)
- Physician assessment of disease activity(Five years after diagnosis)
- BVAS score - Birmingham Vasculitis Activity Score(Five years after diagnosis)