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

ANCA-associated vasculitis

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Other Names:
AAV; Anti-neutrophil cytoplasmic antibody-associated vasculitis; Antineutrophil cytoplasmic antibody-associated vasculitis
Subtypes:
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases (granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis), characterized by destruction and inflammation of small vessels.[1] The clinical signs vary and affect several organs, such as the kidney, stomach, intestine, and lung. Skin lesions, such as purpura and urticaria, result when blood from small vessels leaks under the skin. AAV occurs when neutrophils attack small and medium vessels of the body. The underlying reason for this remains unclear.[2][3] Treatment includes cyclophosphamide,  glucocorticoids and other autoimmune drugs such as rituximab.[1][4] 
Last updated: 4/19/2018

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.

Patient Registry

  • The Autoimmune Registry supports research for ANCA-associated 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.

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

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

  • The HealthWell Foundation provides financial assistance for underinsured patients living with chronic and life-altering conditions. They offer help with drug copayments, deductibles, and health insurance premiums for patients with specific diseases. The disease fund status can change over time, so you may need to check back if funds are not currently available.

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.

In-Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

Videos/Presentations

  • Dr. Ronald Falk presented the annual NIH Astute Clinician Lecture in November 2016 at the NIH Clinical Center. Falk discussed Anti-Neutrophil Cytoplasmic Autoantibodies (ANCA) vasculitis in his lecture titled, "Perspective on Autoimmunity: A View from the ANCA Vasculitis Looking Glass." The lecture is for medical professionals, but may be of interest to anyone who is looking for detailed information on ANCA vasculitis.

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. Lazarus B, John GT, O’Callaghan C & Ranganathan D. Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis. Indian Journal of Nephrology. 2016; 26(2):86–96. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4795442/.
  2. Millet A, Pederzoli-Ribeil M, Guillevin L, Witko-Sarsat V & Mouthon L. Antineutrophil Cytoplasmic Antibody-Associated Vasculitides. Is It Time to Split up the Group?. Ann Rheum Dis. 2013; 72(8):1273-1279. http://www.medscape.com/viewarticle/809119.
  3. What is ANCA Associated Vasculitis (AAV). ANCA associated vasculitis Foundation. https://www.vasculitisfoundation.org/mcm_faq/in-anca-associated-vasculitis-including-granulomatosis-with-polyangiitis-wegeners-gpa-and-microscopic-polyangiitis-mpa-do-increases-in-the-anca-tests-predict-disease-relapse/.
  4. Santana ANC, Viktoria W, Radu HAS & Carmen BSV. Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: update. J. bras. pneumol. December, 2011; 37( 6 ):809-816. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132011000600016&lng=en.