Longitudinal Study for Central Nervous System Vasculitis

NCT ID: NCT05734404

Last Updated: 2026-01-23

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 40 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-03-01
• Study Completion Date: 2030-12-31

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.

Conditions

Central Nervous System Vasculitis; CNS Vasculitis; CNSV; Vasculitis

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Eligibility Criteria

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.

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

The Cleveland Clinic

OTHER

Sponsor Role: collaborator

University of Pennsylvania

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Rula Hajj-Ali, MD

Role: PRINCIPAL_INVESTIGATOR

The Cleveland Clinic

Leonard Calabrese, MD

Role: PRINCIPAL_INVESTIGATOR

The Cleveland Clinic

Peter Merkel, MD

Role: PRINCIPAL_INVESTIGATOR

University of Pennsylvania

Locations

Mayo Clinic

Rochester, Minnesota, United States

Site Status: RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status: RECRUITING

University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status: RECRUITING

Vanderbilt University

Nashville, Tennessee, United States

Site Status: RECRUITING

St. Joseph's Healthcare

Hamilton, , Canada

Site Status: RECRUITING

University of Toronto/Sinai Health

Toronto, , Canada

Site Status: RECRUITING

Countries

United States; Canada

Central Contacts

Carol McAlear

Role: CONTACT

cmcalear@upenn.edu

7813214567

Facility Contacts

Jenni Sletten

Role: primary

Sletten.Jennifer@mayo.edu

Brittany McKnight

Role: primary

mcknigb@ccf.org

Jessica Nguyen

Role: primary

Jessica.Nguyen2@PennMedicine.upenn.edu

Pamela Schlairet

Role: primary

pamela.schlairet@vumc.org

Sandra Messier

Role: primary

smessier@stjosham.on.ca

Nazrana Haq

Role: primary

Nazrana.Haq@sinaihealth.ca

Other Identifiers

VCRC5509

Identifier Type: -

Identifier Source: org_study_id