Evolution of Neuropathies Associated With Necrotizing Vasculitis

Neuropathy Clinical Trial

— NERF-VASC  

Official title:

Evolution of Neuropathies Associated With Necrotizing Vasculitis: Prospective, Non-interventional, Multicentric Study

Clinical Trial Details — Status: Terminated

Administrative data

• NCT number: NCT05164822
• Other study ID #: RC-P0092
• Status: Terminated
• Start date: February 14, 2022
• Est. completion date: October 19, 2022

Study information

• Verified date: September 2023
• Source: Lille Catholic University
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial 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.

Recruitment information / eligibility

• Status: Terminated
• Enrollment: 10
• Est. completion date: October 19, 2022
• Est. primary completion date: October 19, 2022
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Neuropathy
• Peripheral Nervous System Diseases
• Vasculitis

Intervention

Other:
• 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.

Locations

Country	Name	City	State
France	CHU Angers	Angers
France	Hôpital Pellegrin	Bordeaux
France	CH Boulogne-Sur-Mer	Boulogne-sur-Mer
France	Centre Hospitalier Bourg-en-Bresse	Bourg-en-Bresse
France	CHRU Brest	Brest
France	Hôpital Percy	Clamart
France	CHU Clermont-Ferrand	Clermont-Ferrand
France	CHU DIjon-Bourgogne	Dijon
France	Groupe Hospitalier Mutualiste de Grenoble	Grenoble
France	CHRU Lille	Lille
France	CHU Limoges	Limoges
France	Hôpital Edouard Herriot - Hospices Civils de Lyon	Lyon
France	Hôpital Neurologique Pierre Wertheimer - Hospices Civils de Lyon	Lyon
France	AP-HM	Marseille
France	Groupe Hospitalier Sud Ile de France	Melun
France	CHR Metz	Metz
France	UNEOS	Metz
France	Groupe Hospitalier du Havre	Montivilliers
France	CHRU Nancy	Nancy
France	CHU Nantes	Nantes
France	Hôpital de l'Archet	Nice
France	Hôpital Pasteur	Nice
France	CHU Bicêtre	Paris
France	Groupe Hospitalier Paris Saint Joseph	Paris
France	Hôpital Cochin	Paris
France	Hôpital La Pitié Salpétrière	Paris
France	Hôpital Lariboisière	Paris
France	Hôpital Saint-Antoine	Paris
France	Institut Mutualiste Montsouris	Paris
France	Centre Hospitalier Perpignan	Perpignan
France	Hôpital Haut-Lévêque	Pessac
France	CHU de Poitiers	Poitiers
France	Centre Hospitalier de Cornouaille	Quimper
France	CHU Saint-Etienne	Saint-Étienne
France	Centre Hospitalier Saint-Malo	Saint-Malo
France	CHU Strasbourg	Strasbourg
France	Hôpital Foch	Suresnes
France	Hôpital de Rangueil	Toulouse
France	Hôpital Pierre-Paul Riquet	Toulouse
France	Centre Hospitalier Bretagne-Atlantique	Vannes

Sponsors (2)

Lead Sponsor	Collaborator
Lille Catholic University	UNEOS

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Time to remission	The time to remission will be collected. Remission is defined by the absence of new symptoms or lesions due to persistent vasculitis activity and the absence of an inflammatory syndrome	24 months
Primary	Medical Research Council (MRC) score	The muscle scale grades muscle power on a scale of 0 to 5 in relation to the maximum expected for that muscle.; The patient's effort is graded on a scale of 0-5: Grade 5: Muscle contracts normally against full resistance. Grade 4: Muscle strength is reduced but muscle contraction can still move joint against resistance.; Grade 3: Muscle strength is further reduced such that the joint can be moved only against gravity with the examiner's resistance completely removed. As an example, the elbow can be moved from full extension to full flexion starting with the arm hanging down at the side.; Grade 2: Muscle can move only if the resistance of gravity is removed. As an example, the elbow can be fully flexed only if the arm is maintained in a horizontal plane.; Grade 1: Only a trace or flicker of movement is seen or felt in the muscle or fasciculations are observed in the muscle.; Grade 0: No movement is observed.	24 months
Primary	Neuropathy impairment score (NIS)	This score will measure the ankle reflex 0 = normal,; = present with reinforcement,; = absent	24 months
Primary	Rasch-built Overall Disability (R-ODS) Scale	The R-ODS is a linearly weighted scale developed to capture activity and social participation limitations. Score is between 0 and 48 being 0 the worst and 48 the best one.	24 months
Secondary	Correlation coefficient between gravity factors, impact on daily life and dependence	The Correlation coefficient between severity factors, impact on daily life and dependence will be assessed by a mixed model	24 months
Secondary	Response to treatment		3, 6, 9, 12 and 24 months
Secondary	Prevalence of peripheral neurological disorders	Peripheral neurological disorders will be detected by electromyogram	24 months
Secondary	Prevalence of necrotizing vasculitis	Necrotizing vasculitis will be detected by electromyogram	24 months
Secondary	Time to recovery		24 months
Secondary	Medical Research Council (MRC) in patients in remission	The muscle scale grades muscle power on a scale of 0 to 5 in relation to the maximum expected for that muscle.; The patient's effort is graded on a scale of 0-5: Grade 5: Muscle contracts normally against full resistance. Grade 4: Muscle strength is reduced but muscle contraction can still move joint against resistance.; Grade 3: Muscle strength is further reduced such that the joint can be moved only against gravity with the examiner's resistance completely removed. As an example, the elbow can be moved from full extension to full flexion starting with the arm hanging down at the side.; Grade 2: Muscle can move only if the resistance of gravity is removed. As an example, the elbow can be fully flexed only if the arm is maintained in a horizontal plane.; Grade 1: Only a trace or flicker of movement is seen or felt in the muscle or fasciculations are observed in the muscle.; Grade 0: No movement is observed.	24 months
Secondary	Neuropathy impairment score (NIS) in patients in remission	This score will measure the ankle reflex as follows: 0 = normal,; = present with reinforcement,; = absent	24 months
Secondary	Rankin score in patients in remission	This scale is used to categorize the level of functional independence. The scale is between 0 and 5 being 0 without symptoms and 5 meaning severe disability.; 0 No symptoms; No significant disability despite symptoms; able to perform all usual tasks and activities.; Mild disability: unable to perform all previous activities, but able to attend to own affairs without assistance.; Moderate disability: requires assistance, but able to walk without assistance.; Moderately severe disability: unable to walk without assistance and unable to care for own bodily needs without assistance.; Severe disability: bedridden, incontinent and requires constant nursing attention and care.	24 months
Secondary	Birmingham Vasculitis Activity Score (BVAS) in patients in remission	BVAS is a validated tool for assessment of disease activity in patients with many different forms of vasculitis (21-23). The BVAS includes scored items grouped into 9 organ systems which capture a broad spectrum of clinical manifestations from vasculitis. Scores can range from 0 to 63 being 0 the best and 63 the worst.	24 months
Secondary	Visual Analogue Scale (VAS)	Using a ruler, the score is determined by measuring the distance (mm) on the 10-cm line between the "no pain" anchor and the patient's mark, providing a range of scores from 0-10. A higher score indicates greater pain intensity.	24 months