Evolution of Neuropathies Associated With Necrotizing Vasculitis

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.

Conditions

• Necrotizing Vasculitis
• Neuropathy

Interventions

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.

Sponsors & Collaborators

• UNEOS

  collaborator UNKNOWN

• Lille Catholic University

  lead OTHER

Principal Investigators

• François Maurier, MD · UNEOS

Eligibility

Min Age

18 Years

Sex

ALL

Healthy Volunteers

No

Timeline & Regulatory

Start

2022-02-14

Primary Completion

2022-10-19

Completion

2022-10-19

Countries

• France

Study Locations