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.
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
Allocation
NA
Purpose
DIAGNOSTIC
Masking
NONE
Enrollment
100
Blood samples (80 mL) at inclusion, once a year for 5 years, and if relapse or change of treatment
Fecal samples at inclusion
Urinary samples at inclusion, once a year for 5 years, and if relapse or change of treatment
Questionnaires at inclusion, once a year for 5 years, and if relapse or change of treatment
CHRU de Brest - Service de rhumatologie
Brest, Brest, France
RECRUITINGRelapse-free survival of the disease
Relapse-free survival of the disease
Time frame: Five years after diagnosis
death
death
Time frame: Five years after diagnosis
Age
Age
Time frame: Five years after diagnosis
Sex
Sex
Time frame: 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.
Time frame: 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.
Time frame: 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.
Time frame: 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: 1. they have had vasculitis for more than three months of onset of disease. and 2. 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.
Time frame: 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.
Time frame: 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.
Time frame: Five years after diagnosis
Glucocorticoid toxicity index - Glucocorticoid toxicity index during follow-up Glucocorticoid toxicity index
Glucocorticoid toxicity index during follow-up
Time frame: Five years after diagnosis
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