Clinical trial NCT05364892: Biocollection of Patients With ANCA Associated Vasculitis
Official title: 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
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: ANCA-associated Vasculitis
Lead sponsor: University Hospital, Brest
Sponsor class: OTHER
First posted: 2022-05-06
Enrollment: 100
Sex: ALL
Minimum age (years): 18
Maximum age (years): 99
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Allocation: NA
Intervention model: SINGLE_GROUP
Intervention model description: Diagnosis and follow up of ANCA-associated vasculitis
Primary purpose: DIAGNOSTIC

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: Inclusion Criteria:

* 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

Exclusion Criteria:

* Minor
* Patients unable to consent.
* Patients refusing to participate in research
* Patient under legal protection (tutelage, curatorship)
* Pregnant or lactating women
* Hemoglobin (Hb) \< 7g/dL

Primary outcomes:
measure: Relapse-free survival of the disease
timeFrame: Five years after diagnosis
Secondary outcomes:
measure: death
timeFrame: Five years after diagnosis
measure: Age
timeFrame: Five years after diagnosis
measure: Sex
timeFrame: Five years after diagnosis
measure: Physician assessment of disease activity
timeFrame: Five years after diagnosis
measure: Patient assessment of disease activity
timeFrame: Five years after diagnosis
measure: BVAS score - Birmingham Vasculitis Activity Score
timeFrame: Five years after diagnosis
measure: VDI score - Vasculitis Damage Index
timeFrame: Five years after diagnosis
measure: Number of patients with refractory character of the Vasculitis
timeFrame: Five years after diagnosis
measure: HAQ-DI - Health Assessment Questionnaire - Disability Index.
timeFrame: Five years after diagnosis
measure: Glucocorticoid toxicity index - Glucocorticoid toxicity index during follow-up Glucocorticoid toxicity index
timeFrame: Five years after diagnosis
Other outcomes:
Locations:
city: Brest
country: France
facility: CHRU de Brest - Service de rhumatologie
Study officials:
References: