National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Vasculitis

Not a rare disease Not a rare disease
Other Names:
Vasculitis, autoimmune; Angiitis; Autoimmune vasculitis
The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

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.
Last updated: 2/1/2014
Treatment of vasculitis generally depends on the type of vasculitis, the organs affected and the severity in each individual.[1] 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.[1] Commonly used medications have included methotrexate, prednisone and/or cyclophosphamide.[2] 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).[1]

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.[3] See the NIH's news release "Therapy for severe vasculitis shows long-term effectiveness" for more information about this study.
Last updated: 8/5/2013

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

  • 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.
  • The Vasculitis Foundation has a "Find a Physician" map on its website.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Vasculitis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Vasculitis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.
  • 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

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • 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.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Vasculitis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

  • Torpy JM, Lynm C, Glass RM. Vasculitis. The Journal of the American Medical Association. 2007; 289(6):706.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. How is vasculitis treated?. NHLBI. April 1, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/vas/treatment.html. Accessed 8/5/2013.
  2. Vasculitis Treatments. The Johns Hopkins Vasculitis Center. 2013; http://www.hopkinsvasculitis.org/vasculitis-treatments/. Accessed 8/5/2013.
  3. Specks U., et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. August 1, 2013; 369(5):417-427.