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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Vasculitis

*

* Not a rare disease

Other Names for this Disease

  • Angiitis
  • Autoimmune vasculitis
  • Vasculitis, autoimmune
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is vasculitis?

How might vasculitis be treated?

What is vasculitis?

Vasculitis is an inflammation of the vascular system, which includes the veins, arteries, and capillaries. There are many different forms of vasculitis, including temporal arteritis, primary angiitis of the central nervous system, Takayasu disease, periarteritis nodosa, Kawasaki disease, Churg-Strauss syndrome, Wegener granulomatosis, systemic lupus erythematosus, scleroderma, rheumatoid arthritis, Sjogren syndrome, Behcet disease and others. Signs and symptoms of vasculitis vary depending on the form and on the part of the body affected. In general, the cause of vasculitis is thought to be a faulty immune system response. Treatment of vasculitis depends upon the specific diagnosis. Most of forms of vasculitis respond well to steroid drugs. Some may also require treatment with an immunosuppressive drug, such as cyclophosphamide.[1][2]
Last updated: 3/12/2009

How might vasculitis be treated?

Treatment of vasculitis generally depends on the type of vasculitis, the organs affected and the severity in each individual.[3] 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.[3] Commonly used medications have included methotrexate, prednisone and/or cyclophosphamide.[4] 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).[3]

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.[5] 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

References
  1. NINDS Vasculitis Syndromes of the Central and Peripheral Nervous Systems Information Page. National Institute of Neurological Disorders and Stroke. 2008; http://www.ninds.nih.gov/disorders/vasculitis/vasculitis.htm. Accessed 3/12/2009.
  2. What Is Vasculitis?. National Heart, Lung, and Blood Institute. 2006; http://www.nhlbi.nih.gov/health/dci/Diseases/vas/vas_whatis.html. Accessed 3/12/2009.
  3. How is vasculitis treated?. NHLBI. April 1, 2011; http://www.nhlbi.nih.gov/health/health-topics/topics/vas/treatment.html. Accessed 8/5/2013.
  4. Vasculitis Treatments. The Johns Hopkins Vasculitis Center. 2013; http://www.hopkinsvasculitis.org/vasculitis-treatments/. Accessed 8/5/2013.
  5. Specks U., et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. August 1, 2013; 369(5):417-427.


Other Names for this Disease
  • Angiitis
  • Autoimmune vasculitis
  • Vasculitis, autoimmune
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.