Clinical trial NCT05164822: Evolution of Neuropathies Associated With Necrotizing Vasculitis
Official title: Evolution of Neuropathies Associated With Necrotizing Vasculitis: Prospective, Non-interventional, Multicentric Study
Study type: OBSERVATIONAL
Overall status: TERMINATED
Conditions: Necrotizing Vasculitis
Neuropathy
Keywords: Necrotizing Vasculitis
Neuropathy
Score development
Evolution
Lead sponsor: Lille Catholic University
Sponsor class: OTHER
Collaborators: name: UNEOS
First posted: 2021-12-21
Enrollment: 10
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Patient with an initial diagnosis or a relapse of :

- Systemic Vasculitic Neuropathy (SVN): Primary Necrotizing Vasculitis answering the Chapel Hill Consensus Conference criteria associated with a symptomatic Vasculitic Peripheral Neuropathy

Or

- Non Systemic Vasculitic Neuropathy (NSVN): pure symptomatic peripheral neurological impairment without systemic visceral impairment

Brief summary: Necrotizing Vasculitis are inflammatory diseases of the wall of vessels. Neurological damage of the peripheral nerve varies from 7% to 50% of cases depending on the type of Necrotizing Vasculitis.

Peripheral neurological impairment is rarely life threatening (except when associated with other visceral impairment which, in turn, require urgent management with a severity score defined by the Five Factor Score) but impacts the functional outcome by sequelae evaluated by the Vascular Disease Index (VDI).

Four retrospective studies were published with low number of participants, and also mix subgroups of vasculitis Anti-Neutrophil Cytoplasmatic Antibodies (ANCA)+/- GPA (Granulomatosis with polyangiitis), Eosinophilic granulomatosis with polyangiitis (EGPA), Microscopic polyangiitis (MPA), Polyarteritis nodosa (PAN), and Non Systemic Vasculitic Neuropathy (NSVN) and Systemic Vasculitic Neuropathy (SVN).

Overall, management of Necrotizing Vasculitis has evolved significantly over the last two decades, with a dramatic improvement in survival, thanks to new therapeutic strategies and medications. Five-year survival increased from 85% for diagnoses made between 1990 and 1999 to 94.5% for diagnoses made after 2010 Evaluation of relapses of vasculitis, late macro vascular complications, medical-economic evaluation of therapeutic strategies and functional impairment of neuropathies are at the heart of current medical concerns with a view to improve vital and functional prognosis.

Various tests for the evaluation of peripheral neurological damage appear to be relevant tools in vasculitis, although they are not specific: Muscular force scale Medical research council (MRC), Rasch-built overall disability scale (RODS), Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Construction and validation of the chronic acquired polyneuropathy patient-reported index (CAP-PRI), Health-Related Quality of Life (HR QOL), Medical Interview Satisfaction Scale (MISS), Neuropsychological Impairment Scale (NIS) associated to results of repeated Electromyography.

In this study, MRC, NIS and RODS measurements were chosen for their reproducibility and practicality.

In addition to the immediate or relapse mortality factors assessed by the five-factor score (FFS), a functional morbidity score specific to neuropathies related to necrotizing vasculitis must be developed, as well as the determination of the neurosensory disorders and macro-vascular complications. Therefore it is proposed in this observational study to determine the factors that can be predictive of the functional evolution, in order to build a risk score.


Eligibility criteria: Inclusion Criteria:

Adult patient with an initial diagnosis or a relapse of :

- Systemic Vasculitic Neuropathy (SVN): Primary Necrotizing Vasculitis answering the Chapel Hill Consensus Conference criteria associated with a symptomatic Vasculitic Peripheral Neuropathy

Or

- Non Systemic Vasculitic Neuropathy (NSVN): pure symptomatic peripheral neurological impairment without systemic visceral impairment

Exclusion Criteria:

* Symptomatic peripheral neurological impairment not attributable exclusively to primary necrotizing vasculitis
* Diabetes
* Chronic ethylism
* AL (immunoglobulin light chain) amyloidosis or TTR (transthyretin)
* Genetic neuropathies
* Toxic neuropathies
* Sequelae of sciatica
* Opposition to participation in the study
* Lack of social security
* Under guardianship or curatorship

Primary outcomes:
measure: Time to remission
timeFrame: 24 months
measure: Medical Research Council (MRC) score
timeFrame: 24 months
measure: Neuropathy impairment score (NIS)
timeFrame: 24 months
measure: Rasch-built Overall Disability (R-ODS) Scale
timeFrame: 24 months
Secondary outcomes:
measure: Correlation coefficient between gravity factors, impact on daily life and dependence
timeFrame: 24 months
measure: Response to treatment
timeFrame: 3, 6, 9, 12 and 24 months
measure: Prevalence of peripheral neurological disorders
timeFrame: 24 months
measure: Prevalence of necrotizing vasculitis
timeFrame: 24 months
measure: Time to recovery
timeFrame: 24 months
measure: Medical Research Council (MRC) in patients in remission
timeFrame: 24 months
measure: Neuropathy impairment score (NIS) in patients in remission
timeFrame: 24 months
measure: Rankin score in patients in remission
timeFrame: 24 months
measure: Birmingham Vasculitis Activity Score (BVAS) in patients in remission
timeFrame: 24 months
measure: Visual Analogue Scale (VAS)
timeFrame: 24 months
Other outcomes:
Locations:
city: Angers
country: France
facility: CHU Angers
city: Bordeaux
country: France
facility: Hôpital Pellegrin
city: Boulogne-sur-Mer
country: France
facility: CH Boulogne-Sur-Mer
city: Bourg-en-Bresse
country: France
facility: Centre Hospitalier Bourg-en-Bresse
city: Brest
country: France
facility: CHRU Brest
city: Clamart
country: France
facility: Hôpital Percy
city: Clermont-Ferrand
country: France
facility: CHU Clermont-Ferrand
city: Dijon
country: France
facility: CHU DIjon-Bourgogne
city: Grenoble
country: France
facility: Groupe Hospitalier Mutualiste de Grenoble
city: Lille
country: France
facility: CHRU Lille
city: Limoges
country: France
facility: CHU Limoges
city: Lyon
country: France
facility: Hôpital Edouard Herriot - Hospices Civils de Lyon
city: Lyon
country: France
facility: Hôpital Neurologique Pierre Wertheimer - Hospices Civils de Lyon
city: Marseille
country: France
facility: AP-HM
city: Melun
country: France
facility: Groupe Hospitalier Sud Ile de France
city: Metz
country: France
facility: CHR Metz
city: Metz
country: France
facility: UNEOS
city: Montivilliers
country: France
facility: Groupe Hospitalier du Havre
city: Nancy
country: France
facility: CHRU Nancy
city: Nantes
country: France
facility: CHU Nantes
city: Nice
country: France
facility: Hôpital de l'Archet
city: Nice
country: France
facility: Hôpital Pasteur
city: Paris
country: France
facility: CHU Bicêtre
city: Paris
country: France
facility: Groupe Hospitalier Paris Saint Joseph
city: Paris
country: France
facility: Hôpital Cochin
city: Paris
country: France
facility: Hôpital La Pitié Salpétrière
city: Paris
country: France
facility: Hôpital Lariboisière
city: Paris
country: France
facility: Hôpital Saint-Antoine
city: Paris
country: France
facility: Institut Mutualiste Montsouris
city: Perpignan
country: France
facility: Centre Hospitalier Perpignan
city: Pessac
country: France
facility: Hôpital Haut-Lévêque
city: Poitiers
country: France
facility: CHU de Poitiers
city: Quimper
country: France
facility: Centre Hospitalier de Cornouaille
city: Saint-Malo
country: France
facility: Centre Hospitalier Saint-Malo
city: Saint-Étienne
country: France
facility: CHU Saint-Etienne
city: Strasbourg
country: France
facility: CHU Strasbourg
city: Suresnes
country: France
facility: Hôpital Foch
city: Toulouse
country: France
facility: Hôpital de Rangueil
city: Toulouse
country: France
facility: Hôpital Pierre-Paul Riquet
city: Vannes
country: France
facility: Centre Hospitalier Bretagne-Atlantique
Study officials:
affiliation: UNEOS
name: François Maurier, MD
role: STUDY_CHAIR
References: