Longitudinal Study for Central Nervous System Vasculitis

Vasculitis Clinical Trial

Official title:

Longitudinal Study for Central Nervous System Vasculitis

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05734404
• Other study ID #: VCRC5509
• Status: Recruiting
• Start date: March 1, 2023
• Est. completion date: December 31, 2026

Study information

• Verified date: February 2024
• Source: University of Pennsylvania
• Contact: Carol McAlear
• Phone: 7813214567
• Email: cmcalear[@]upenn.edu
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 40
• Est. completion date: December 31, 2026
• Est. primary completion date: December 31, 2026
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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.

Related Conditions & MeSH terms

• Central Nervous System Vasculitis
• CNS Vasculitis
• Vasculitis
• Vasculitis, Central Nervous System

Locations

Country	Name	City	State
Canada	St. Joseph's Healthcare	Hamilton
Canada	University of Toronto/Sinai Health	Toronto
United States	Cleveland Clinic	Cleveland	Ohio
United States	Vanderbilt University	Nashville	Tennessee
United States	University of Pennsylvania	Philadelphia	Pennsylvania

Sponsors (2)

Lead Sponsor	Collaborator
University of Pennsylvania	The Cleveland Clinic

Countries where clinical trial is conducted

United States,  Canada, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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.	Study completion; from baseline through month 36.
Secondary	PROMIS Questionnaires	Questions will ask about Cognition function on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about depression on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about fatigue on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about Ability to participate in social roles and activities on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about Upper extremity function on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about lower extremity function on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about Global Health (physical) on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36
Secondary	PROMIS Questionnaires	Questions will ask about Global Health (mental) on the PROMIS short form.	Baseline, month 3, month 6, month 9, month 12, month 18, month 24, month 30, month 36