Evolution of Neuropathies Associated With Necrotizing Vasculitis

NCT ID: NCT05164822

Last Updated: 2023-09-14

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

TERMINATED

Total Enrollment

10 participants

Study Classification

OBSERVATIONAL

Study Start Date

2022-02-14

Study Completion Date

2022-10-19

Brief Summary

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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.

Detailed Description

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Conditions

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Necrotizing Vasculitis Neuropathy

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Necrotizing Vasculitis

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

Necrotizing Vasculitis

Intervention Type OTHER

Pain scale Diagnosis of necrotizing vasculitis and peripheral neuropathy, Clinical characteristics of the disease, Biological analysis, Treatment and evolution of the disease,

Questionnaires :

Neuropathy Impairment Score (NIS) sensitive sub-questionnaire that measures extremity sensitivity.

MRC (Medical Research Council), which evaluates three motor functions on each member.

RODS questionnaire on the relationship between your daily activities and your health.

BVAS (Birmingham Vasculitis Activity Score) disease activity questionnaire. VDI (Vasculitis Damage Index) questionnaire evaluates the sequelae of the disease.

Rankin Score modified to measure the degree of disability

Two tests will be performed:

EMG (electromyogram) to evaluate the electrical activity of the muscles, Walking Test of 10 meters which aims to measure the time taken to travel 10 meters.

Interventions

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Necrotizing Vasculitis

Pain scale Diagnosis of necrotizing vasculitis and peripheral neuropathy, Clinical characteristics of the disease, Biological analysis, Treatment and evolution of the disease,

Questionnaires :

Neuropathy Impairment Score (NIS) sensitive sub-questionnaire that measures extremity sensitivity.

MRC (Medical Research Council), which evaluates three motor functions on each member.

RODS questionnaire on the relationship between your daily activities and your health.

BVAS (Birmingham Vasculitis Activity Score) disease activity questionnaire. VDI (Vasculitis Damage Index) questionnaire evaluates the sequelae of the disease.

Rankin Score modified to measure the degree of disability

Two tests will be performed:

EMG (electromyogram) to evaluate the electrical activity of the muscles, Walking Test of 10 meters which aims to measure the time taken to travel 10 meters.

Intervention Type OTHER

Eligibility Criteria

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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
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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UNEOS

UNKNOWN

Sponsor Role collaborator

Lille Catholic University

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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François Maurier, MD

Role: STUDY_CHAIR

UNEOS

Locations

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CHU Angers

Angers, , France

Site Status

Hôpital Pellegrin

Bordeaux, , France

Site Status

CH Boulogne-Sur-Mer

Boulogne-sur-Mer, , France

Site Status

Centre Hospitalier Bourg-en-Bresse

Bourg-en-Bresse, , France

Site Status

CHRU Brest

Brest, , France

Site Status

Hôpital Percy

Clamart, , France

Site Status

CHU Clermont-Ferrand

Clermont-Ferrand, , France

Site Status

CHU DIjon-Bourgogne

Dijon, , France

Site Status

Groupe Hospitalier Mutualiste de Grenoble

Grenoble, , France

Site Status

CHRU Lille

Lille, , France

Site Status

CHU Limoges

Limoges, , France

Site Status

Hôpital Edouard Herriot - Hospices Civils de Lyon

Lyon, , France

Site Status

Hôpital Neurologique Pierre Wertheimer - Hospices Civils de Lyon

Lyon, , France

Site Status

AP-HM

Marseille, , France

Site Status

Groupe Hospitalier Sud Ile de France

Melun, , France

Site Status

CHR Metz

Metz, , France

Site Status

UNEOS

Metz, , France

Site Status

Groupe Hospitalier du Havre

Montivilliers, , France

Site Status

CHRU Nancy

Nancy, , France

Site Status

CHU Nantes

Nantes, , France

Site Status

Hôpital de l'Archet

Nice, , France

Site Status

Hôpital Pasteur

Nice, , France

Site Status

CHU Bicêtre

Paris, , France

Site Status

Groupe Hospitalier Paris Saint Joseph

Paris, , France

Site Status

Hôpital Cochin

Paris, , France

Site Status

Hôpital La Pitié Salpétrière

Paris, , France

Site Status

Hôpital Lariboisière

Paris, , France

Site Status

Hôpital Saint-Antoine

Paris, , France

Site Status

Institut Mutualiste Montsouris

Paris, , France

Site Status

Centre Hospitalier Perpignan

Perpignan, , France

Site Status

Hôpital Haut-Lévêque

Pessac, , France

Site Status

CHU de Poitiers

Poitiers, , France

Site Status

Centre Hospitalier de Cornouaille

Quimper, , France

Site Status

CHU Saint-Etienne

Saint-Etienne, , France

Site Status

Centre Hospitalier Saint-Malo

St-Malo, , France

Site Status

CHU Strasbourg

Strasbourg, , France

Site Status

Hôpital Foch

Suresnes, , France

Site Status

Hôpital de Rangueil

Toulouse, , France

Site Status

Hôpital Pierre-Paul Riquet

Toulouse, , France

Site Status

Centre Hospitalier Bretagne-Atlantique

Vannes, , France

Site Status

Countries

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France

Other Identifiers

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RC-P0092

Identifier Type: -

Identifier Source: org_study_id

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