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

Overview

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.

Interventions

• Other Blood samples (80 mL)
  Blood samples (80 mL) at inclusion, once a year for 5 years, and if relapse or change of treatment
• Other Fecal samples
  Fecal samples at inclusion
• Other Urinary sample (20-40 mL)
  Urinary samples at inclusion, once a year for 5 years, and if relapse or change of treatment
• Other Questionnaires
  Questionnaires at inclusion, once a year for 5 years, and if relapse or change of treatment

Primary outcome measures

• Relapse-free survival of the disease [Time frame: Five years after diagnosis]

Secondary outcome measures (10)

• death [Time frame: Five years after diagnosis]
• Age [Time frame: Five years after diagnosis]
• Sex [Time frame: Five years after diagnosis]
• Physician assessment of disease activity [Time frame: Five years after diagnosis]
• Patient assessment of disease activity [Time frame: Five years after diagnosis]
• BVAS score - Birmingham Vasculitis Activity Score [Time frame: Five years after diagnosis]
• VDI score - Vasculitis Damage Index [Time frame: Five years after diagnosis]
• Number of patients with refractory character of the Vasculitis [Time frame: Five years after diagnosis]
• HAQ-DI - Health Assessment Questionnaire - Disability Index. [Time frame: Five years after diagnosis]
• Glucocorticoid toxicity index - Glucocorticoid toxicity index during follow-up Glucocorticoid toxicity index [Time frame: Five years after diagnosis]

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

Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.

Healthy volunteers: Yes

Study design

Allocation

N/A

Model

Single group

Masking

Open label

Primary purpose

Diagnostic

Study locations

France · 1 center

• CHRU de Brest - Service de rhumatologie — Brest

Identifiers

NCT: NCT05364892 · 29BRC20.0233

Primary sources (government registries)

• ClinicalTrials.gov (US NIH)

View this study on ClinicalTrials.gov ↗