Central Nervous System Vasculitis, CNSV, CNS Vasculitis, Vasculitis
Conditions
Brief summary
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.
Interventions
None listed
Sponsors
University of Pennsylvania
The Cleveland Clinic
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
i. Diagnostic criteria for CNSV 1. The presence of an acquired and otherwise unexplained neurologic deficit 2. The presence of either classic angiographic or histopathologic features of angiitis in the CNS 3. 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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Evaluate the pathogenesis of central nervous system vasculitis using disease history. | Study completion; from baseline through month 36. | To study the manner of development of disease in patients affected with central nervous system vasculitis. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| PROMIS Questionnaires | Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36 | Questions will ask about Cognition function on the PROMIS short form. |
Countries
Canada, United States
Contacts
CONTACTCarol McAlear
PRINCIPAL_INVESTIGATORRula Hajj-Ali, MD
The Cleveland Clinic
PRINCIPAL_INVESTIGATORLeonard Calabrese, MD
The Cleveland Clinic
PRINCIPAL_INVESTIGATORPeter Merkel, MD
University of Pennsylvania
Outcome results
None listed