Necrotizing Vasculitis, Neuropathy
Conditions
Keywords
Necrotizing Vasculitis, Neuropathy, Score development, Evolution
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.
Interventions
OTHERNecrotizing 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.
Sponsors
UNEOS
Lille Catholic University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Time to remission | 24 months | 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 |
| Medical Research Council (MRC) score | 24 months | 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. |
| Neuropathy impairment score (NIS) | 24 months | This score will measure the ankle reflex 0 = normal, 1. = present with reinforcement, 2. = absent |
| Rasch-built Overall Disability (R-ODS) Scale | 24 months | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Time to recovery | 24 months | — |
| Medical Research Council (MRC) in patients in remission | 24 months | 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. |
| Neuropathy impairment score (NIS) in patients in remission | 24 months | This score will measure the ankle reflex as follows: 0 = normal, 1. = present with reinforcement, 2. = absent |
| Correlation coefficient between gravity factors, impact on daily life and dependence | 24 months | The Correlation coefficient between severity factors, impact on daily life and dependence will be assessed by a mixed model |
| Birmingham Vasculitis Activity Score (BVAS) in patients in remission | 24 months | 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. |
| Visual Analogue Scale (VAS) | 24 months | 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. |
| Rankin score in patients in remission | 24 months | 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 1. No significant disability despite symptoms; able to perform all usual tasks and activities. 2. Mild disability: unable to perform all previous activities, but able to attend to own affairs without assistance. 3. Moderate disability: requires assistance, but able to walk without assistance. 4. Moderately severe disability: unable to walk without assistance and unable to care for own bodily needs without assistance. 5. Severe disability: bedridden, incontinent and requires constant nursing attention and care. |
| Response to treatment | 3, 6, 9, 12 and 24 months | — |
| Prevalence of peripheral neurological disorders | 24 months | Peripheral neurological disorders will be detected by electromyogram |
| Prevalence of necrotizing vasculitis | 24 months | Necrotizing vasculitis will be detected by electromyogram |
Countries
France
Outcome results
None listed