Vasculitis
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Description
The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
Orpha Number: 52759
Definition
Vasculitis represents a clinically heterogenous group of diseases of multifactorial etiology characterized by inflammation of either large-sized vessels (large-vessel vasculitis, e.g. Giant-cell arteritis and Takayasu arteritis; see these terms), medium-sized vessels (medium-vessel vasculitis e.g. polyarteritis nodosa and Kawasaki disease; see these terms), or small- sized vessels (small-vessel vasculitis, e.g. granulomatosis with polyangiitis, microscopic polyangiitis, immunoglobulin A vasculitis, and cutaneous leukocytoclastic angiitis; see these terms). Vasculitis occurs at any age, may be acute or chronic, and manifests with general symptoms such as fever, weight loss and fatigue, as well as more specific clinical signs depending on the type of vessels and organs affected. The degree of severity is variable, ranging from life or sight threatening disease (e.g. Behçet disease, see this term) to relatively minor skin disease.
Visit the Orphanet disease page for more resources.
Source: GARD Last updated on 05-01-20
How might vasculitis be treated?
Treatment of vasculitis generally depends on the type of vasculitis, the organs affected and the severity in each individual. The main goal of treatment is to reduce inflammation in the affected blood vessels. Individuals with mild vasculitis may only need over-the-counter pain medications, while those with severe vasculitis are often treated with prescription medications including corticosteroids and/or cytotoxic medicines that reduce or stop the immune response causing the inflammation. Commonly used medications have included methotrexate, prednisone and/or cyclophosphamide. In rare cases, surgery may be needed. Other treatments may be used for certain types of vasculitis (for example, high-dose aspirin and immune globulin for Kawasaki syndrome).
A study by U. Specks et al. published in August of 2013 in the New England Journal of Medicine found that a medication called rituximab is as effective as cyclophosphamide and glucocorticoids for ANCA-associated vasculitis; the authors reported that rituximab also requires a shorter treatment duration. For the patients in this study, administration of rituximab once a week for 4 weeks, followed by placebo, was as effective in the treatment of severe ANCA-associated vasculitis as conventional immunosuppressive therapy administered for 18 months. See the NIH's news release "Therapy for severe vasculitis shows long-term effectiveness" for more information about this study.
Last updated on 05-01-20
Healthcare Resources
Vasculitis Foundation
The Vasculitis Foundation has a "Find a Physician" map on its website.
Last updated on 04-27-20
Vasculitis - Medical consultants Vasculitis foundation
If your physician would like to review your case with a vasculitis specialist, the Vasculitis Foundation lists vasculitis medical consultants that may be able to speak with your healthcare provider. Click on the link above to learn more.
Last updated on 04-27-20
In-Depth Information
MeSH®
MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
Last updated on 04-27-20
Other Conferences
All Star Vasculitis Patient Symposium
All Star Vasculitis Patient Symposium
July 30, 2010 - August 1, 2010
Location: Westin Long Beach Hotel in Long Beach, California
Description: Topics will cover all the types of vasculitis and will
concentrate on the advances in medical treatments, research, and quality of
life of patients. The meeting attracts vasculitis patients, family members,
and medical vasculitis experts in the world.
Last updated on 04-27-20
Patient Registry
RDCRN - Vasculitis Clinical Research Consortium (VCRC)
The Vasculitis Clinical Research Consortium (VCRC) is an integrated group of academic medical centers, patient support organizations, and clinical research resources dedicated to conducting clinical research in different forms of vasculitis. The VCRC has a contact registry for patients who wish to be contacted about clinical research opportunities and updates on the progress of the VCRC research projects.
For more information on the registry see: https://www.rarediseasesnetwork.org/cms/vcrc/About-Us
Last updated on 04-27-20
Selected Full-Text Journal Articles
JAMA article - vasculitis
Torpy JM, Lynm C, Glass RM. Vasculitis. The Journal of the American Medical Association. 2007; 289(6):706.
Last updated on 04-27-20
Where To Start
National Institute of Neurological Disorders and Stroke (NINDS)
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
Last updated on 04-27-20
John's hopkins vasculitis center - vasculitis info
The Johns Hopkins Vasculitis CenterWeb site has an information page on this topic. Click on the link above to view this information.
Last updated on 04-27-20
Kansas City, MO, 64188, United States Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: https://www.vasculitisfoundation.org/contact-us-2/ Url: https://www.vasculitisfoundation.org/ Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
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