Antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis, or AAV, causes inflammation of the blood vessels and can affect many parts of the body, including multiple organs. Because of this, AAV care is usually managed by a team of medical specialists.
Many people receive a consultation or are treated at vasculitis centers or academic hospitals, where doctors have specific experience with AAV. No matter where you get treatment, having a coordinated care team is essential. It’s one of the reasons outcomes for AAV are much better today than they were in the past. While new treatments offer real hope, AAV remains a condition that requires long-term management. Even after reaching remission, you will need regular follow-ups to stay in remission, monitor medication side effects, and prevent relapse.
Left untreated, AAV can lead to serious complications, including kidney failure, structural lung damage, and an increased risk of cardiovascular disease. And the treatment itself, which often includes long-term steroid use, comes with its own health risks. Given these different aspects of AAV, building the right care team is a crucial part of managing the disease.
Here are a few members to consider, depending on your overall health and what parts of your body are affected:
Rheumatologist In most cases, the lead doctor will be a rheumatologist, says Robert Spiera, MD, a rheumatologist at the Hospital for Special Surgery in New York City. Rheumatologists are specialists in inflammatory autoimmune conditions. As the lead doctor, they will likely make most of the therapy decisions, which often involves a combination of immunosuppressants and anti-inflammatory medications. They will work to get you in remission and keep you there.
“This disease is in our domain. We deal with systemic autoimmune diseases. We are very comfortable with the medicines, and we are trained in multi systems. We are not just thinking of kidneys or just thinking of nerves or just joints or just lungs,” says Dr. Spiera. “And we are good at monitoring these medicines and addressing their side effects.”
Primary Care Physician Your primary care physician may have been your first stop when you began to have symptoms of AAV. Their job would be to refer you to a specialist — in most cases, a rheumatologist. But their involvement does not stop there. They are critical to your care team.
They can help monitor medication side effects, such as high blood pressure or hypoglycemia, watch for signs of infection (since you are at higher risk), and assess your mental health.
Nephrologist The organ most commonly affected by AAV are the kidneys, especially with the type of AAV called microscopic polyangiitis. A nephrologist specializes in the management of kidney disease. If you only have kidney involvement, a nephrologist may be your lead doctor. If you require a kidney biopsy, that would also be in the nephrologist’s domain.
Pulmonologist You will need a pulmonologist if you have symptoms such as hemorrhaging in the lungs. “Pulmonologists are often involved if you have endobronchial disease or if there is a lung nodule from the disease,” says Spiera. And they might help as part of the support team if you ever end up in intensive care with a flare-up. In rare cases, they might do stenting procedures to bypass areas of inflammation, he says.
Otolaryngologist One type of AAV, called granulomatosis with polyangiitis (GPA) affects the ears, nose, and sinuses. An otolaryngologist, or ENT, may be involved in confirming the diagnosis of GPA with a biopsy. They are also essential in managing sinonasal (nose and sinuses) involvement in GPA, Spiera says. In some cases, an ENT will perform surgery for symptom relief, such as placing tubes in the ears. The ENT may also help by widening the trachea if it’s been narrowed by the disease.
Neurologist A neurologist may be consulted if there is nerve damage or dysfunction. According to Spiera, a neurologist can be helpful in providing an objective opinion about neurological damage, often evaluating a person every six months.
Dermatologist Some possible symptoms of AAV are itchy, red bumps, blisters, or painful ulcers. You may see a dermatologist to get a biopsy to confirm the diagnosis of the disease, as well as manage any skin symptoms.
Ophthalmologist AAV often causes inflammatory eye disease from swelling of the tiny blood vessels in the eye. Inflammation of the white part of the eye is most common. An ophthalmologist can help identify eye damage and manage therapy complications, such as glaucoma. In severe cases, an ophthalmologist might repair eye damage through surgery, such as grafts, patching, and tissue gluing.
The Takeaway
ANCA-associated vasculitis (AAV) is often treated by a team of doctors, because it affects multiple organs and systems in the body.
In most cases, a rheumatologist will head up your medical team, as they are specialists in autoimmune diseases.
You may also have other specialists involved in your care, such as a nephrologist, ophthalmologist, otolaryngologist, or pulmonologist, depending on where AAV has affected your body.
EDITORIAL SOURCES
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Resources
Shum M et al. Primary Care Physicians Play a Crucial Role in Diagnosing and Managing Rare Eosinophilic Diseases: HES and EGPA. Frontiers in Medicine. June 24, 2025.
Padoan R et al. Ear, Nose, and Throat in ANCA-Associated Vasculitis: A Comprehensive Review. Vessel Plus. May 23, 2021.
Macarie SS et al. Therapy of Ocular Complications in ANCA+ Associated Vasculitis. Romanian Journal of Ophthalmology. January-March 2021.
Alexa Meara, MD, is an assistant professor of immunology and rheumatology at The Ohio State University. She maintains a multidisciplinary vasculitis clinic and supervises a longitudinal registry of lupus nephritis and vasculitis patients. Her clinical research is in improving patient–physician communication. She is involved in the medical school and the Lead-Serve-Inspire (LSI) curriculum and serves on the medical school admissions committee; she also teaches multiple aspects of the Part One curriculum. Her interests in medical-education research include remediation and work with struggling learners.
Dr. Meara received her medical degree from Georgetown University School of Medicine in Washington, DC. She completed her internal medicine training at East Carolina University (ECU) at Vidant Medical Center in Greenville, North Carolina, then spent two more years at ECU, first as chief resident in internal medicine, then as the associate training program director for internal medicine. She pursued further training in rheumatology at The Ohio State University in Columbus, completing a four-year clinical and research fellowshipthere in 2015.
Nina Wasserman is a journalist with more than a decade of experience interviewing people and writing on a variety of topics, including health, medicine, business, and faith, as well as human interest stories. Wasserman also home-schools her two children in New Jersey and teaches writing to middle school students. Her passion is foraging for mushrooms and edible plants in the woods, a practice that contributes to her health and wellness.
