Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis

NCT02257866 | Recruiting

• 2 other identifiers: 14020014-AR-0200
• Study Type: observational
• Target: 4,000
• Locations: 1 country
• Sites: 1

Brief Summary

Background: \- Vasculitis is a group of diseases that inflame and damage blood vessels and tissue. It can cause many medical problems. Few tests can diagnose the disease, and none can reliably predict a relapse. Researchers want to study people s genes and follow people over time to see how the disease affects them. Objective: \- To learn the signs, symptoms, imaging tests, genetic markers, and blood tests that can help identify people with vasculitis and predict what will happen to them over time. Eligibility:

• People age 3 and older who have or are thought to have vasculitis, or are related to someone with it.
• Healthy volunteers. Design:
• Participants will be evaluated by a doctor who has expertise caring for patients with vasculitis.
• Participants will give a blood sample. Some will give a urine sample.
• Some participants may have brushings or biopsies taken from the inside lining of the nose.
• Images of participants blood vessels may be taken using scans. For some scans, participants will lie on a table that moves in and out of a cylinder that takes pictures. For some scans, a contrast agent may be injected into an arm vein. Other scans may use a radioactive form of sugar. Healthy minors will not have scans.
• Some participants will answer questionnaires. - Some participants will have their tests done at NIH. Others will have their doctor take the blood, saliva, or cheek swab samples and send them to NIH.
• Some participants will have one visit lasting 1-2 (but sometimes up to 4) days. Some participants may have follow-up visits every 3 - 6 months, indefinitely.

Conditions

Takayasu's Arteritis; Giant Cell Arteritis; Polyarteritis Nodosa; Relapsing Polychondritis; ANCA-Associated Vasculitis

Keywords

Polyarteritis Nodosa; Takayasu's Arthritis; Giant Cell Arthritis; Relapsing Polychondritis; Antineutrophil Cytoplasmic Antibodies; Natural History

Outcome Measures

Primary Outcomes (1)

• To study the pathogenesis of patients affected with idiopathic systemic vasculitis, including clinical, radiographic, immunological, and genetic characteristics of the disease

  To study the pathogenesis of patients affected with idiopathic systemic vasculitis, including clinical, radiographic, immunological, and genetic characteristics of the disease

  end of study

Study Arms (2)

Healthy Volunteers

Patients without known auto immune diseases

Vasculitis

Patients with known or suspected vasculitis age 5 or older

Eligibility Criteria

• Age: 3 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

idiopathic systemic vasculitis in children and adults

You may qualify if:

• SUBJECTS WITH VASCULITIS
• Subjects who fulfill modified versions of the 1990 American College of Rheumatology (ACR) Classification Criteria for GPA31 and PAN
• Subjects who fulfill the 1990 ACR Classification Criteria for EGPA, GCA, and TAK
• Subjects who fulfill the 2012 Chapel Hill Nomenclature definition for MPA
• Subjects with other suspected systemic or single-organ vasculitides
• HEALTHY VOLUNTEERS
• Volunteers able to provide consent, or in the case of minors, assent

You may not qualify if:

• SUBJECTS WITH VASCULITIS:
• Subjects less than 3 years of age
• Inability to provide consent, or in the case of minors, assent
• Subjects with bleeding diathesis or on anticoagulant medications (e.g. coumadin, heparin, clopidogrel but not including aspirin or NSAIDs) are excluded from participation in nasal brushing or biopsy studies
• HEALTHY VOLUNTEERS
• Volunteers less than 3 years of age
• Diagnosis of vasculitis or other autoimmune/autoinflamamtory disease, including systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, mixed connective tissue disease or any overlap autoimmune syndrome
• Pregnant (by history of last menstrual period) or breast feeding subjects
• Subjects with bleeding diathesis or taking anticoagulant medications (eg coumadin, heparin, clopidogrel but not including aspirin or NSAIDs) are excluded from participating in nasal brushing studies

Sponsors & Collaborators

• National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS): lead

Study Sites (1)

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

RECRUITING

Related Publications (6)

• Carmona-Rivera C, Purmalek MM, Moore E, Waldman M, Walter PJ, Garraffo HM, Phillips KA, Preston KL, Graf J, Kaplan MJ, Grayson PC. A role for muscarinic receptors in neutrophil extracellular trap formation and levamisole-induced autoimmunity. JCI Insight. 2017 Feb 9;2(3):e89780. doi: 10.1172/jci.insight.89780.

  PMID: 28194438 BACKGROUND

• Grayson PC, Alehashemi S, Bagheri AA, Civelek AC, Cupps TR, Kaplan MJ, Malayeri AA, Merkel PA, Novakovich E, Bluemke DA, Ahlman MA. 18 F-Fluorodeoxyglucose-Positron Emission Tomography As an Imaging Biomarker in a Prospective, Longitudinal Cohort of Patients With Large Vessel Vasculitis. Arthritis Rheumatol. 2018 Mar;70(3):439-449. doi: 10.1002/art.40379. Epub 2018 Feb 6.

  PMID: 29145713 BACKGROUND

• Quinn KA, Ahlman MA, Malayeri AA, Marko J, Civelek AC, Rosenblum JS, Bagheri AA, Merkel PA, Novakovich E, Grayson PC. Comparison of magnetic resonance angiography and 18F-fluorodeoxyglucose positron emission tomography in large-vessel vasculitis. Ann Rheum Dis. 2018 Aug;77(8):1165-1171. doi: 10.1136/annrheumdis-2018-213102. Epub 2018 Apr 17.

  PMID: 29666047 BACKGROUND

• Rose E, Ferrada MA, Quinn KA, Goodspeed W, Arnaud L, Sharma A, Yoshifuji H, Kim J, Allen C, Sirajuddin A, Chen M, Grayson PC. Physician Global Assessment as a Disease Activity Measure for Relapsing Polychondritis. Arthritis Care Res (Hoboken). 2022 Aug;74(8):1269-1276. doi: 10.1002/acr.24574. Epub 2022 Apr 29.

  PMID: 33544969 DERIVED

• Rimland CA, Quinn KA, Rosenblum JS, Schwartz MN, Bates Gribbons K, Novakovich E, Sreih AG, Merkel PA, Ahlman MA, Grayson PC. Outcome Measures in Large Vessel Vasculitis: Relationship Between Patient-, Physician-, Imaging-, and Laboratory-Based Assessments. Arthritis Care Res (Hoboken). 2020 Sep;72(9):1296-1304. doi: 10.1002/acr.24117.

  PMID: 31785185 DERIVED

• Michailidou D, Rosenblum JS, Rimland CA, Marko J, Ahlman MA, Grayson PC. Clinical symptoms and associated vascular imaging findings in Takayasu's arteritis compared to giant cell arteritis. Ann Rheum Dis. 2020 Feb;79(2):262-267. doi: 10.1136/annrheumdis-2019-216145. Epub 2019 Oct 24.

  PMID: 31649025 DERIVED

Related Links

• NIH Clinical Center Detailed Web Page

MeSH Terms

Conditions

Takayasu Arteritis; Giant Cell Arteritis; Polyarteritis Nodosa; Polychondritis, Relapsing; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Condition Hierarchy (Ancestors)

Aortic Arch Syndromes; Aortic Diseases; Vascular Diseases; Cardiovascular Diseases; Arteritis; Vasculitis; Skin Diseases, Vascular; Skin Diseases; Skin and Connective Tissue Diseases; Vasculitis, Central Nervous System; Autoimmune Diseases of the Nervous System; Nervous System Diseases; Cerebrovascular Disorders; Brain Diseases; Central Nervous System Diseases; Autoimmune Diseases; Immune System Diseases; Systemic Vasculitis; Cartilage Diseases; Musculoskeletal Diseases; Connective Tissue Diseases

Study Officials

• Peter C Grayson, M.D.

  National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Shanni Liu

CONTACT

(240) 992-2440; shanni.liu@nih.gov

Peter C Grayson, M.D.

CONTACT

(240) 731-4558; peter.grayson@nih.gov

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: October 4, 2014
• First Posted: October 7, 2014
• Study Start: September 29, 2014
• Primary Completion (Estimated): January 1, 2050
• Study Completion (Estimated): January 1, 2050
• Last Updated: March 20, 2026

Record last verified: 2026-02-12

Locations

US (1)