Longitudinal Study for Central Nervous System Vasculitis
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- This is an observational study: the protocol does not assign a study treatment.
- Who it may be relevant to
- Registry conditions: Central Nervous System Vasculitis, CNS Vasculitis, CNSV, Vasculitis. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- United States, Canada
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Overview
Primary central nervous system vasculitis (CNSV) is a potentially fatal, single-organ vasculitis that often involves a spectrum of neurologic complications, including strokes, cognitive and speech impairment, visual loss, dementia, and encephalopathy. The purpose of this study is to establish a research cohort to investigate the disease process, treatments, and patient outcomes in CNSV.
Detailed description
Primary central nervous system vasculitis (CNSV) is a potentially fatal, single-organ vasculitis that often involves a spectrum of neurologic complications, including strokes, cognitive and speech impairment, visual loss, dementia, and encephalopathy. Originally described in the early 1950s, research in the disease has been slow to progress, largely limited by the rarity of the disease and the lack of biologic materials for basic and translational investigations. To date, the bulk of research in the field has been clinical, largely consisting of retrospective case series, predominately from single sites, focusing on correlations among clinical, imaging, and histopathology data. Collectively these series suggest the existence of clinically-relevant subsets. Such studies, while enhancing our knowledge of this rare disease, are limited by small numbers of patients (especially those confirmed by histopathology), and the near absence of detailed investigations into the biomolecular and immunopathogenic basis of the disease.
Development of this longitudinal cohort and multicenter approach offers the potential to generate new insights, generate new questions, and further our understanding of its natural history, pathophysiology, genetic basis, and discovery of biomarkers to assess disease activity and prognosis.
Study visits will occur every 3 months for this first year, and then annually for two more years. Blood collection will occur at every visit. A physical exam and medical and medication history will at every visit; also, participants will be asked to complete several questionnaires to assess disease activity, health status, and tobacco, alcohol, and drug use.
Primary outcome measures
- Evaluate the pathogenesis of central nervous system vasculitis using disease history. [Time frame: Study completion; from baseline through month 36.]
Secondary outcome measures (8)
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
- PROMIS Questionnaires [Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36]
Eligibility criteria
Inclusion criteria
i. Diagnostic criteria for CNSV
- The presence of an acquired and otherwise unexplained neurologic deficit
- The presence of either classic angiographic or histopathologic features of angiitis in the CNS
- No evidence of systemic vasculitis or any condition that could cause the angiographic or pathologic features
ii. ≥18 years of age
Exclusion criteria
i. Unwilling to sign informed consent.
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
United States · 4 centers
- Mayo Clinic — Rochester
- Cleveland Clinic — Cleveland
- University of Pennsylvania — Philadelphia
- Vanderbilt University — Nashville
Canada · 2 centers
- St. Joseph's Healthcare — Hamilton
- University of Toronto/Sinai Health — Toronto
Identifiers
NCT: NCT05734404 · VCRC5509