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ANCA-associated vasculitis and its impact on patients and families


In October 2022, RARE Revolution and Amgen co-hosted a RARE Rev-inar to discuss ANCA-associated vasculitis and its impact on patients and families. The insightful session featured a nephrologist, a rheumatologist, a patient and the director of a patient support group

By Geoff Case

The insightful session featured a nephrologist, a rheumatologist, a patient and the director of a PAG on ANCA-associated vasculitis and its impact.
Dr Duvuru Geetha, professor of clinical medicine in the division of nephrology at Johns Hopkins University School of Medicine and associate director of the Johns Hopkins Vasculitis Center
The insightful session featured a nephrologist, a rheumatologist, a patient and the director of a PAG on ANCA-associated vasculitis and its impact.
Joyce Kullman, executive director at Vasculitis Foundation
The insightful session featured a nephrologist, a rheumatologist, a patient and the director of a PAG on ANCA-associated vasculitis and its impact.
Glen Massie, a patient advocate living with ANCA-associated vasculitis

Dr Alvin Wells, director, department of rheumatology, Advocate Aurora Medical Group, Franklin, Wisconsin, United States
Geoff Case, digital editor, RARE Revolution Magazine
Brian Tumminello, senior medical director, medical affairs, Amgen

1. The healthcare professional perspective

What is ANCA-associated vasculitis (AAV)?

Vasculitis is a general term that refers to inflammation of the blood vessels and damage to the endothelium and tissue. ANCA-associated vasculitis (AAV) is an umbrella term for a group of these diseases.1 They are autoimmune diseases: the majority of patients have a circulating protein called ANCA (antineutrophil cytoplasmic antibody) that attacks the body’s own cells.1-4 The two most common types of AAV are:1

  • granulomatosis with polyangiitis (GPA)
  • microscopic polyangiitis (MPA)

“AAV can pretty much affect any blood vessel in the body—predominantly, small- and medium-sized blood vessels.1 Symptoms vary from person to person and really depend on what organ is affected,” Dr Geetha explains.5,6 AAV commonly affects the kidneys, lungs, skin, ENT (ear, nose and throat), nerves and joints.2

“AAV is a serious but treatable disease, so early diagnosis and treatment are critical. Treatment often involves using steroids with other medications that suppress your immune system and help to reduce the inflammation,” she says.1,7

It’s common for patients with AAV to experience cycles of relapse (getting worse) and remission (getting better).5 Dr Geetha emphasises the importance of visiting the doctor regularly for blood and urine tests that can monitor disease activity.

What makes AAV difficult to diagnose?

Dr Geetha believes that there are three factors that make AAV a difficult condition to diagnose:

  • its rarity
  • its symptoms mimic those of other conditions and may be non-specific
  • its impact on multiple systems in the body

The first factor leading to delays in diagnosis is the rarity of AAV.1 The annual incidence of AAV is 13 to ~20 cases per million in Europe.8 The annual incidence of AAV in the US is about 7,377 cases per 100,000, with a prevalence of about 94,116 per 100,000.9,10 “Unless you have expertise with AAV, you’re not going to think about the condition. In some of the studies that looked at the median time to diagnosis, it was about seven months,” she says.11 A delay in diagnosis can have serious consequences for patients, depending on which organ is involved and how active the disease is.1

The second factor slowing the diagnosis of AAV is that symptoms may mimic those of other common diseases.11 Symptoms may also be non-specific (not due to any single known cause).6 “Patients can just be feeling lousy; they can have fevers, night sweats, weight loss… Oftentimes, rheumatologic diseases, infections, cancers or allergies can present the same way, so it’s really hard for a primary care physician who has not seen a case of vasculitis to think about AAV.”

What symptoms do people with AAV have at diagnosis?

In Dr Geetha’s experience, the majority of patients come to doctors saying they just don’t feel well, perhaps reporting loss of appetite, drowsiness (lethargy) or weight loss. They may report additional symptoms depending on where the disease is active. For instance, if there’s sinus involvement, they might report nose bleeds, or if they have lung involvement, they might report shortness of breath, or even coughing of blood. If there is eye involvement, they can have painful eyes, red eyes or loss of vision. If their skin is affected, they may have a rash. If the nerves are affected, people can have tingling, numbness and loss of movement of hands or feet. With kidney involvement, people might have no symptoms until late stage, so the first signs of disease may come from blood and urine tests.

The third factor that can delay a diagnosis is that AAV affects multiple organs, so some patients may be treated by different specialists, most commonly a rheumatologist, nephrologist, pulmonologist or ENT doctor (otolaryngologist).1,12,13 As Dr Geetha believes, these specialists might focus on treating the symptoms affecting a particular organ without taking account of other signs and symptoms affecting other parts of the body. If those signs and symptoms were considered and the right tests were done promptly, a diagnosis could soon be made. Better education about AAV would address this knowledge gap and speed up diagnosis.

“Collaboration among specialists is really important… If someone comes with a systemic disease affecting more than one organ, we have to think about vasculitis in addition to everything else. Collaboration is also key in managing the complications that are related to the disease and its treatment.”

Do people with AAV know enough about it; how can they find out more?

Patients with AAV aren’t getting the right information about their disease. A 2014 study compared the informational needs of AAV patients. It concluded that AAV patients would like to receive information about their disease, treatment regimens and side effects, and that they would prefer to get this information directly from their physician. It also found that patient education should be a fundamental part of their care.14

In a 2022 study, ten people with AAV were interviewed to find out what information they thought was needed. The researchers identified five themes: patients need oral and written information to be combined; they need information about living with the disease; they need information about symptoms and indications of relapse; they need psychological support when receiving information about the disease; they need a peer to share information with.15

As Dr Geetha shares, there are many organisations that support and educate patients, such as Vasculitis Foundation (in the United States), Vasculitis Foundation Canada and Vasculitis UK. Their local support groups bring people with AAV together, so that they can learn from one another’s experiences.

What does remission mean in AAV?

For Dr Wells, a patient is in remission from AAV when they have few clinical symptoms, few flare-ups and they are on the lowest dose of corticosteroids possible.

The challenge for rheumatologists is controlling the disease without having side effects due to medication. “We know for every one milligram increase in corticosteroids you end up with side effects,” he says. These can affect many organs in the body, potentially causing issues such as osteoporosis, cataracts or diabetes.16

Dr Wells monitors his patients carefully, holding virtual clinics every three months and in-person appointments every six to nine months. Between those times, patients are monitored through bloodwork, urine tests and imaging, to help ensure that the disease is kept under control.

Clinical guidelines used to measure disease activity, especially in clinical trials, can be quite cumbersome for use in a clinic, Dr Wells believes. However, he describes the Birmingham Vasculitis Activity Score (BVAS) as a useful “yardstick” for assessing how active the disease is in a patient.1 In his experience, the results from patients’ regular tests can be combined with insights from talking to the patient to understand what is happening.

“In those 15 minutes, I ask how are you doing? Have you met the goals that you want? Are there any new problems or symptoms going on? And we go from there.”

As Dr Wells believes, setting goals and forming a path is an important part of the conversation when clinicians and patients discuss remission. “When we talk about remission, we start with the patient and ask, ‘what do you want to be able to do that you can’t do now?’ and we make that our goal.”

Dr Wells thinks of an AAV patient as being in complete remission if they have no clinical signs of disease and their imaging (CT scans of the kidney or lungs, for example) and lab tests are almost normal.

What tests are used when checking for remission?

There are several tests that can be done to monitor a patient who has AAV. These include taking a complete blood count (CBC), measuring liver and kidney function, and checking for general inflammation.17

Dr Wells explains the need for the CBC: “Because some of our medicines are immunosuppressive, they’re going to lower the blood count.18,19 We aim to get the white cell count down because that drives the disease, but we don’t want to go too low because we have to think about COVID and infections.”

The liver function test is for two reasons. First, because AAV can attack the liver.20 Second, autoimmune diseases can overlap, so a patient with AAV may develop autoimmune hepatitis too.21

The kidneys are monitored to see how well they are doing their job of excreting waste from the body.22 This is done through testing how much creatinine (a waste product from the muscles) is in the blood or urine.1,23 “We want to make sure that creatinine stays normal. It can be compromised just from the inflammation from the disease itself.”

How is disease severity defined? Is localised disease severe?

In AAV, disease that is localised can be severe.13,24,25 In Dr Wells’ experience, somebody with GPA might have sinus problems which require treatment with several medications. One such patient might perceive their disease as severely active whereas another might think of it as more moderate. “That’s where the physician comes in, to balance that out and see which patient needs aggressive therapy,” Dr Wells says.

2. Lived experience

What was your diagnostic journey like?

Glen Massie was diagnosed with GPA in 2012 after experiencing symptoms that had “smouldered” for a couple of years. His was a difficult diagnostic journey in which he had to take the lead in searching for answers, consulting several doctors.

In the autumn of the previous year, Glen had developed a serious ear infection, extreme pain in his back and the feeling that something was moving in his chest. At first, his doctor thought that he had pulled a muscle, but in fact he had a pleural effusion (a build-up of excess fluid around the lungs).26 This happened four times and required a procedure known as a thoracentesis to drain the fluid.27

“While this was going on, I developed excruciating joint pains. I recall lying in a recliner thinking this is the worst thing that’s ever happened to me. I praise God I had the use of one of my arms, but because of the joint pain in the rest of my body, I really couldn’t do anything but sit in a chair.”

Soon afterwards, a petechial rash (pinpoint spots) developed on Glen’s legs which gradually spread to his torso.28 His doctor took a skin biopsy to investigate the rash, but while Glen waited for the results, he had an alveolar haemorrhage (bleeding in the alveolar spaces in the lung) which eventually put him into respiratory failure.29 Prompt immunosuppressive therapy might have prevented this, he says. Glen needed to be airlifted to a specialist clinic, where he was put on a ventilator for two weeks.

What are your current symptoms?

“I describe my disease activity as a ‘smoulder’. It seems like there’s always something going on. It’s not fully involved. But the smoulder presents some smaller challenges—daily challenges.”

Over the many years since Glen’s diagnosis, sinusitis has been a consistent challenge, and joint pain has recurred, particularly in the colder months. Glen also has a perforated sinus, and the continued growth of the perforation worries him. He fears that he may ultimately develop a nasal deformity (a saddle nose deformity) and that his ability to breathe will be affected.25

What impact does AAV have on your overall quality of life?

All of these health issues have impacted Glen’s quality of life, forcing him to come to terms with a “new normal”. He has been taking corticosteroids for 11 years and is on a maintenance dosage now, but even that is damaging, he says. His treatment with corticosteroids has caused diabetes, vision changes, weight gain, osteoporosis and emotional swings.

The osteoporosis has been difficult for Glen. He thinks it is the joint pain from that—rather than from disease activity directly—that limits his ability to do the things that he would normally enjoy doing.

“I would love nothing more than to get on the floor with my grandchildren and play; I can’t get out and run in the yard with them. I enjoy camping, fishing and things like that. I can’t do those type of things. GPA has limited my ability to do the things that I would normally enjoy doing.”

Glen has found that his mood swings depend on the dosage of the corticosteroids that he is on at a given time. The combined effect of these drugs and the pandemic was a “perfect storm” that took a heavy toll on his mental well-being. He had to contend not only with the emotional side effects of the drugs but also with the isolation from friends and family, who are so important in his life. Although he still experiences difficult days, he feels that the mild antidepressants prescribed by his rheumatologist are helping.

Glen is probably as close to remission now as he has ever been, having overcome his alveolar haemorrhage and respiratory issues. But he says that it doesn’t feel like he is close to remission because of the growing perforation in his nose—a sign that the disease is still active. He requires daily medication and infusions twice a year. He would consider getting off the maintenance dosage of corticosteroids a great success.

How important is joint decision-making?

Glen has found that the rheumatologist and specialists at his clinic have a strong understanding of the challenges he faces, but he believes that local doctors struggle to fully understand the issues.

He really values his relationship with his rheumatologist, who has encouraged him to let her know of any concerns he has and not dismiss them as symptoms of ageing. “She says, all the time, if you’re having an issue notify me and we’ll talk it through together. Don’t assume it’s this or that—let us help you figure that out…”

“I’ve been so appreciative of that openness: there are a lot of times I would have made some bad calls if I had left it up to me to determine what was going on.”

What is the biggest unmet need for people with AAV?

Glen believes that the biggest unmet need for patients like himself is access to local doctors that understand vasculitis. When his diagnostic journey started, his own doctor wasn’t able to give him the answers he needed, so he consulted another one. That doctor had a refreshing approach that earned Glen’s confidence.

 “He said ‘I know nothing about what’s going on with you but I’m willing to learn it with you.’ He was willing to put in the time, and we worked together to find the specialist I needed.”

3. Patient support groups: Vasculitis Foundation

How does the Vasculitis Foundation support patients?

The Vasculitis Foundation plays an important role in supporting patients with AAV in three main areas:

  1. Helping patients with vasculitis receive an earlier diagnosis.
  2. Improving access to expert medical care.
  3. Providing educational resources that patients need to help them manage AAV.

Joyce says that the focus needs to be on managing AAV because it is a chronic condition.

“Education and support are the heart and soul of our organisation.”

One of the biggest challenges for the foundation has been keeping up with changes in how people learn. When the pandemic started in 2020, the foundation quickly revamped its website and increased its digital presence so that it could keep meeting its goals. For example, its in-person conferences became virtual learning sessions.

The foundation’s Road to Wellness webinars feature medical experts sharing their knowledge, responding to questions that patients have submitted. Patients often want to learn which of their symptoms are caused by their AAV and which are caused by their treatment. They also want to learn how to manage these symptoms.

Joyce observed “a huge decline” in mental health wellness during the pandemic, when isolation meant that patients couldn’t interact with family and friends. The Vasculitis Foundation has weekly and monthly support groups to bring people together.

“Just to be together, even virtually, has been really helpful to people.”

In 2021, Vasculitis Foundation helped launch the American College of Rheumatology treatment guidelines for six forms of vasculitis, including EGPA, MPA and GPA. These recommendations provide guidance regarding the evaluation and management of patients, including diagnostic strategies and the use of drugs and surgical interventions. Joyce is hopeful that these guidelines will help local doctors and rheumatologists better understand how to diagnose and treat the disease; not everyone can get to specialist centres.

The Vasculitis Foundation’s Victory over Vasculitis initiative, a campaign led by patient ambassadors, helps patients to have hope and set goals for themselves to define what their own victory over vasculitis would look like. The programme has three components: physical, mental health and self-advocacy. The physical component varies according to the patient. “It is not about everybody running a marathon—it may be going to the grocery store by yourself and getting groceries. We’re trying to meet patients where they are and help them have hope,” Joyce says.

“Years ago, vasculitis was a death sentence, but it isn’t now. We need to help patients have hope that they can have lives that may not be 100%, but may be close to that—even living with vasculitis.

How does the Vasculitis Foundation support research and patient engagement?

The Vasculitis Foundation’s Research Program funds small pilot studies with grants lasting a year or two. “Over the past 20 years we’ve funded over three million dollars in research with 53 studies worldwide,” Joyce says.

As well as funding research, Vasculitis Foundation also encourages patients to participate in research opportunities—anything from focus groups to clinical trials.

“If our patients don’t participate in research, it’s not going to get done. We’re never going to get the answers that we need to the questions we all have,” Joyce says.

The foundation is focusing on encouraging younger physicians and scientists through research grants. “We provide grants where they can study novel ideas. Our goal is to bring them into vasculitis to help them get established as independent researchers outside of their mentor’s field,” Joyce says.

The Vasculitis Foundation also funds fellowships to train young physician-investigators to come into the field of vasculitis research. Its one- or two-year fellowship grants are aimed at encouraging them not just to learn about clinical care but also to explore the idea of doing research in vasculitis.

“The goal behind the fellowship and the young investigator program is to create more experts in vasculitis who can help our patients no matter where they live in the world.”

Joyce hopes that in the future patients will be able to access expert care locally and not have to travel, as Glen and many other patients have needed to.

What is the Vasculitis Patient-Powered Research Network (VPPRN)?

The Vasculitis Patient-Powered Research Network (VPPRN) is an international research network of patients, scientists, clinicians, advocates and family members. It seeks to improve healthcare and quality of life for patients with vasculitis through patient-centred clinical research.

Since its start in 2014, the VPPRN has enrolled over 3,000 patient partners in 17 studies, which have covered 18 types of vasculitis.30 Each study explores a research question that patients are interested in and involves them in the whole research process—from study design to data collection, to review and even publication of the results.

“I’m so happy that patients are involved in research now. Study design can make or break a trial so it’s very exciting to see our patients engaged.”

Recent examples of VPPRN research studies:

  • the impact of vasculitis-related skin involvement on patients’ quality of life
  • the length of time required for a diagnosis
  • the impact of COVID-19 (a collaboration with autoimmune disease groups)
  • the effect of vasculitis on pregnancy

“The COVID-19 study was a very interesting process. We learned a lot and it also made patients feel empowered because they were trying to solve the questions not just sitting back in their bubbles: they were actually participating in research.”

What are the challenges in raising awareness of AAV?

One of the biggest challenges for rare disease support groups is raising awareness of the condition. “We don’t have anybody famous with the disease, so we have to raise awareness one person at a time,” Joyce says.

The foundation’s approach to raising awareness and sharing information has evolved over time as new platforms have become available. “We used to hand people brochures, and then we built the website, and then we used social media,” Joyce says.

Online platforms offer “a tremendous opportunity” to raise awareness, but it can be hard to be heard. “People are bombarded every day with ‘news’—we know so much about some people’s lives that we have no business knowing,” Joyce says. “We seem to focus on light-hearted matters as opposed to serious things that impact people’s lives. Why don’t people know about vasculitis? It’s such a critically important issue.”

Another challenge is accommodating patients’ varied preferences for how they receive information: “We have people who will read journal articles, but we have other patients who watch 30-second Tik Tok videos.”

If you are interested in learning more about ANCA-associated vasculitis, please visit: 

⦁ National Kidney Foundation at

⦁ Vasculitis Foundation at

⦁ National Organization for Rare Disorders at


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