Biocollection of Patients With ANCA Associated Vasculitis (ANCA)

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

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.

Study Overview

• Status: Recruiting
• Conditions: ANCA-associated Vasculitis
• Intervention / Treatment: Other: Blood samples (80 mL); Other: Fecal samples; Other: Urinary sample (20-40 mL); Other: Questionnaires

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.

• Study Type: Interventional
• Enrollment (Anticipated): 100
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Divi CORNEC; Phone Number: +33 (0)2 98 34 72 64; Email: divi.cornec@chu-brest.fr

Study Locations

• France
  • Brest, France, 29200
    • Recruiting
    • CHRU de Brest - Service de rhumatologie
    • Contact: Divi CORNEC; Phone Number: 02 98 34 72 64; Email: divi.cornec@chu-brest.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 99 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Diagnostic
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

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.

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

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Relapse-free survival of the disease	Relapse-free survival of the disease	Five years after diagnosis

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
death	death	Five years after diagnosis
Age	Age	Five years after diagnosis
Sex	Sex	Five years after diagnosis
Physician assessment of disease activity	Disease activity will be assessed on a scale from 0 to 100, considering the pain and the impact on daily life. A higher score means a worse outcome.	Five years after diagnosis
Patient assessment of disease activity	Disease activity will be assessed on a scale from 0 to 100, considering the pain and the impact on daily life. A higher score means a worse outcome.	Five years after diagnosis
BVAS score - Birmingham Vasculitis Activity Score	Questionnaire listing 56 symptoms divided into nine organ/systems classes, plus an "other" section. For each item, the assessor evaluates if it is present and attribuable to the active vasculitis or not. A higher score means a worse outcome.	Five years after diagnosis
VDI score - Vasculitis Damage Index	This is for recording organ damage that has occurred in patients since the onset of vasculitis, and over 3 months. Record features of active disease using the Birmingham Vasculitis Activity Score (BVAS). A new patient should usually have a VDI score of zero, unless: they have had vasculitis for more than three months of onset of disease. and; the damage has developed or become worse since the onset of vasculitis Questionnaire listing 64 symptoms divided into eleven organ/systems classes. For each item, the assessor evaluates if it is present over 3 months and attribuable to the active vasculitis or not. A higher score means a worse outcome.	Five years after diagnosis
Number of patients with refractory character of the Vasculitis	Number of patients for whome a secondary decision to intensify immunosupressive treatment in the first year of treatment (increased corticosteroid dosage, introduction of another immunosupressor outside the scheduled at the end of the initial assessment) has been taken.	Five years after diagnosis
HAQ-DI - Health Assessment Questionnaire - Disability Index.	Evolution of HAQ-DI during follow-up 8 fields questionnaire (DRESSING & GROOMING, ARISING, EATING, WALKING, HYGENE, REACH, GRIP, Other activites), scaled from 0 to 3, 3 meaning a worse outcome.	Five years after diagnosis
Glucocorticoid toxicity index - Glucocorticoid toxicity index during follow-up Glucocorticoid toxicity index	Glucocorticoid toxicity index during follow-up	Five years after diagnosis

Collaborators and Investigators

• Sponsor: University Hospital, Brest

Study record dates

Study Major Dates

• Study Start (Actual): October 27, 2022
• Primary Completion (Anticipated): October 27, 2032
• Study Completion (Anticipated): October 27, 2032

Study Registration Dates

• First Submitted: December 21, 2020
• First Submitted That Met QC Criteria: May 4, 2022
• First Posted (Actual): May 6, 2022

Study Record Updates

• Last Update Posted (Actual): October 31, 2022
• Last Update Submitted That Met QC Criteria: October 28, 2022
• Last Verified: October 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Immune System Diseases; Autoimmune Diseases; Systemic Vasculitis; Vasculitis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Other Study ID Numbers: 29BRC20.0233

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Yes
• IPD Plan Description: All collected data that underlie results in a publication
• IPD Sharing Time Frame: Data will be available after the publication of result and ending fifteen years following the last visit of the last patient
• IPD Sharing Access Criteria: Data access requests will be reviewed by the internal committee of Brest UH. Requestors will be required to sign and complete a data access agreement
• IPD Sharing Supporting Information Type: Study Protocol

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No