An observational study in Central Nervous System Vasculitis, CNS Vasculitis and CNSV, sponsored by University of Pennsylvania. Recruiting at 6 sites in 2 countries. Open to participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2026-01-23.
Sponsored by University of Pennsylvania · Observational
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.
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.
170 studies on the registry are indexed under Vasculitis; 46 are open to participants now.
This study's planned enrollment of 40 is below the median of 147 across 77 observational studies indexed under Vasculitis.
Browse Vasculitis studies →University of Pennsylvania is the lead sponsor of 1,668 studies on the registry; 235 are open to participants now.
Of its 158 completed or terminated interventional studies of FDA-regulated products, 109 (69%) have results posted.
Counted across the registry records on this site, refreshed daily.
Individuals with central nervous system vasculitis (CNSV). Enrollment will be sequential and participants will have disease in various stages and of different duration.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
i. Diagnostic criteria for CNSV
ii. ≥18 years of age
Exclusion Criteria:
i. Unwilling to sign .
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Evaluate the pathogenesis of central nervous system vasculitis using disease history.
To study the manner of development of disease in patients affected with central nervous system vasculitis.
Time frame: Study completion; from baseline through month 36.
PROMIS Questionnaires
Questions will ask about Cognition function on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about depression on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about fatigue on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about Ability to participate in social roles and activities on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about Upper extremity function on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about lower extremity function on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about Global Health (physical) on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
PROMIS Questionnaires
Questions will ask about Global Health (mental) on the PROMIS short form.
Time frame: Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Plan to share: No
No publications or documents are linked to this record.
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