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

May 28, 2026 updated by: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

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.

Study Overview

Status

Recruiting

Conditions

Detailed Description

The purpose of this protocol is to study the natural history of idiopathic systemic vasculitis in children and adults. The idiopathic vasculitides are a group of rare, systemic diseases involving inflammation of arteries and other tissue with resulting organ- and life-threatening disease courses. The different forms of idiopathic vasculitis are typically classified based upon the predominant size of the arteries affected in each condition, including small vessel vasculitis [granulomatosis with polyangiitis (GPA, Wegener s), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss)]; medium vessel vasculitis [polyarteritis nodosa (PAN)]; and large vessel vasculitis [giant cell arteritis (GCA), Takayasu s arteritis (TAK), idiopathic aortitis (IA)]. Although patients with each type of vasculitis manifest disease-specific aspects of illness, there are substantial disease and treatment burdens common to patients with vasculitis. For each type of idiopathic vasculitis, the disease course is often chronic, relapse is common and unpredictable, organ and tissue damage can accrue over time, new symptoms can occur late into the disease course, and treatment is often associated with toxicity and serious side effects.

The goals of this natural history protocol are to establish a cohort of pediatric and adult patients with vasculitis to prospectively evaluate the signs and symptoms, imaging findings, and blood and tissue biomarkers associated with pathogenesis and disease outcomes. In the small vessel vasculitides, where considerable progress has been made towards identifying pathologic mechanisms of disease, we will focus on elucidating the pathogenic role of neutrophils, selected biomarkers such as SERPINA1, and novel candidate biomarkers in circulating blood and at local tissue sites including the nasal mucosa. In the medium and large vessel vasculitides, we will identify novel candidate biomarkers for disease pathogenesis and outcomes and develop disease activity indices that incorporate existing and novel clinical, laboratory, genomic, and imaging biomarkers. For all types of vasculitis, a goal of the protocol is to identify patients for possible entry into future treatment studies.

Patients enrolled in this protocol will undergo a history, physical examination, and laboratory evaluation. Since vasculitis is multi-system disease with variable patterns of organ involvement, patients may undergo further comprehensive evaluation of a particular affected organ system when clinically indicated. Peripheral blood samples will be collected from affected patients, unrelated healthy volunteers matched for age, sex, and ethnicity whenever possible, and in some cases unaffected family members to help identify and study the genes involved in vasculitis and their functions. We may ask some patients to undergo nasal biopsy and additional imaging studies for research purposes. For a small number of patients and family members, we may ask permission to perform whole genome or exome sequencing. Successful completion of these studies will improve our understanding of disease pathogenesis.

Study Type

Observational

Enrollment (Estimated)

4000

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

  • United States
    • Maryland
      • Bethesda, Maryland, United States, 20892
        • Recruiting
        • National Institutes of Health Clinical Center
        • Contact:
          • For more information at the NIH Clinical Center contact Office of Patient Recruitment (OPR)
          • Phone Number: TTY dial 711 800-411-1222
          • Email: ccopr@nih.gov

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

3 years and older (Child, Adult, Older Adult)

Accepts Healthy Volunteers

Yes

Sampling Method

Non-Probability Sample

Study Population

idiopathic systemic vasculitis in children and adults

Description

  • INCLUSION CRITERIA:

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

EXCLUSION CRITERIA:

SUBJECTS WITH VASCULITIS:

  • Subjects less than 3 years of age
  • Active malignancy, infection, or any medical condition that in the opinion of the investigator would warrant exclusion
  • 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
  • Active malignancy, infection, or any medical condition that in the opinion of the investigator would warrant exclusion
  • 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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort
Healthy Volunteers
Patients without known auto immune diseases
Vasculitis
Patients with known or suspected vasculitis age 5 or older

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
To study the pathogenesis of patients affected with idiopathic systemic vasculitis, including clinical, radiographic, immunological, and genetic characteristics of the disease
Time Frame: end of study
To study the pathogenesis of patients affected with idiopathic systemic vasculitis, including clinical, radiographic, immunological, and genetic characteristics of the disease
end of study

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

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

Investigators

  • Principal Investigator: Peter C Grayson, M.D., National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

September 29, 2014

Primary Completion (Estimated)

January 1, 2050

Study Completion (Estimated)

January 1, 2050

Study Registration Dates

First Submitted

October 4, 2014

First Submitted That Met QC Criteria

October 4, 2014

First Posted (Estimated)

October 7, 2014

Study Record Updates

Last Update Posted (Actual)

May 29, 2026

Last Update Submitted That Met QC Criteria

May 28, 2026

Last Verified

February 12, 2026

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • 140200
  • 14-AR-0200

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

IPD Plan Description

Protocol is silent about IPD sharing.

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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