NCT02257866

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

Recruiting now Last updated 20 March 2026

What this trial tests

trial in Takayasu's Arteritis in 4,000 participants. Currently enrolling.

Timeline

29 September 2014

Primary endpoint
1 January 2050

1 January 2050

Quick facts

Lead sponsor	National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
Status	Recruiting now
Study type	OBSERVATIONAL
Enrollment	4,000
Start date	29 September 2014
Primary completion	1 January 2050
Estimated completion	1 January 2050
Sites	1 location across United States

Conditions studied

• Takayasu's Arteritis — all drugs for Takayasu's Arteritis →
• Giant Cell Arteritis — all drugs for Giant Cell Arteritis →
• Polyarteritis Nodosa — all drugs for Polyarteritis Nodosa →
• Relapsing Polychondritis — all drugs for Relapsing Polychondritis →

Sponsor

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

Who can join

3 and older, any sex, with Takayasu's Arteritis or Giant Cell Arteritis. Patients with the condition only — healthy volunteers not accepted.

Sponsor's own description

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.

Publications & conference data

8 peer-reviewed publications reference this trial (live from Europe PMC):

1. Somatic Mutations in <i>UBA1</i> and Severe Adult-Onset Autoinflammatory Disease.
   Beck DB, Ferrada MA, Sikora KA, Ombrello AK, et al · · 2020 · cited 844× · PMID 33108101 · DOI 10.1056/nejmoa2026834
2. <sup>18</sup> F-Fluorodeoxyglucose-Positron Emission Tomography As an Imaging Biomarker in a Prospective, Longitudinal Cohort of Patients With Large Vessel Vasculitis.
   Grayson PC, Alehashemi S, Bagheri AA, Civelek AC, et al · · 2018 · cited 235× · PMID 29145713 · DOI 10.1002/art.40379
3. Benign and malignant hematologic manifestations in patients with VEXAS syndrome due to somatic mutations in UBA1.
   Obiorah IE, Obiorah IE, Patel BA, Groarke EM, et al · · 2021 · cited 177× · PMID 34427584 · DOI 10.1182/bloodadvances.2021004976
4. Deficiency of adenosine deaminase 2 triggers adenosine-mediated NETosis and TNF production in patients with DADA2.
   Carmona-Rivera C, Khaznadar SS, Shwin KW, Irizarry-Caro JA, et al · · 2019 · cited 125× · PMID 31015188 · DOI 10.1182/blood.2018892752
5. Comparison of magnetic resonance angiography and <sup>18</sup>F-fluorodeoxyglucose positron emission tomography in large-vessel vasculitis.
   Quinn KA, Ahlman MA, Malayeri AA, Marko J, et al · · 2018 · cited 93× · PMID 29666047 · DOI 10.1136/annrheumdis-2018-213102
6. Patterns of Arterial Disease in Takayasu Arteritis and Giant Cell Arteritis.
   Gribbons KB, Ponte C, Carette S, Craven A, et al · · 2020 · cited 78× · PMID 31444857 · DOI 10.1002/acr.24055
7. Use of 18F-fluorodeoxyglucose positron emission tomography to monitor tocilizumab effect on vascular inflammation in giant cell arteritis.
   Quinn KA, Quinn KA, Dashora H, Novakovich E, et al · · 2021 · cited 50× · PMID 33369678 · DOI 10.1093/rheumatology/keaa894
8. Effect of Treatment on Imaging, Clinical, and Serologic Assessments of Disease Activity in Large-vessel Vasculitis.
   Banerjee S, Quinn KA, Gribbons KB, Rosenblum JS, et al · · 2020 · cited 46× · PMID 30877209 · DOI 10.3899/jrheum.181222