Rula Hajj-Ali, MD, is a rheumatologist, the associate director of the Center for Vasculitis Care and Research at the Cleveland Clinic, and professor of medicine at the Cleveland Clinic Lerner College of Medicine at Case Western Reserve University in Cleveland, Ohio.
ANCA-associated vasculitis (AAV) is a group of rare autoimmune diseases that includes:
- Eosinophilic granulomatosis with polyangiitis (EGPA)
- Granulomatosis with polyangiitis (GPA)
- Microscopic polyangiitis (MPA)
ANCA stands for “anti-neutrophil cytoplasmic antibodies.” These are proteins that hijack neutrophils, white blood cells made by your immune system. Those neutrophils then attack your small blood vessels, leading to ongoing inflammation that can cause harm to organs throughout your body.
There isn’t a cure for AAV. But it can be managed with medication so people with this condition can live a normal life. Still, the condition is challenging, particularly because its rarity keeps it off many doctors’ radars. That can delay diagnosis and treatment.
We spoke to rheumatologist Rula Hajj-Ali, MD, the associate director of the Center for Vasculitis Care and Research at the Cleveland Clinic in Ohio, to get more insights into this condition.
What’s going on in the body when you have ANCA-associated vasculitis?
The immune system, which normally we have to protect us from germs, is mistakenly attacking the blood vessels and specifically causing inflammation in the small blood vessels throughout the body.
Why is it so important to diagnose and treat AAV as early as possible?
This is a disease that can affect multiple organs such as the kidneys, the lungs, the skin, the nerves, the sinuses, and the eyes. If it’s not treated, it can cause serious and permanent damage in these organs.
What are the earliest symptoms?
The first symptoms that happen with AAV can be very tricky to recognize because they can feel like common illnesses, such as the flu. The symptoms can be fever, fatigue, weight loss, muscle aches, and joint pain. Kidney problems usually are not symptomatic. Unfortunately, people can have a significant degree of kidney involvement without any symptoms until it is really very advanced. That’s not the case when the lungs are affected. Symptoms like cough, shortness of breath, or even coughing up blood can develop. Other possible symptoms are sinusitis, nosebleeds, inflammation, and swelling of the eyes, as well as skin and nerve problems.
If you have flu-like symptoms, how can you tell there’s something more serious happening?
When you have a lot of symptoms here and there, it can be very hard to diagnose. And it also can take a long time for symptoms to accumulate. I have patients who have had chronic sinus disease for years before other symptoms happened. It’s hard to just put this process together as a patient, so I advise that you see your doctor if there’s something that’s not going away within two to three weeks, as most common illnesses like the flu get better by then.
Many doctors may not suspect AAV. How can I convince my doctor to take a closer look?
Doctors often don’t recognize it. Both specialists and primary care doctors may tend to deal with one episode of one, acute symptom rather than putting everything together. Be an advocate for yourself. Have a list of all of your symptoms. It’s really helpful for your physician to see that there’s not just one thing going on, but that multiple things are going on. Fortunately, awareness is growing among both providers and patients, and we’re seeing patients getting diagnosed earlier and earlier.
Once you suspect AAV, how is it diagnosed?
Testing includes a blood test that looks for ANCA autoantibodies, a marker in the blood that’s present in about 95% of patients with ANCA-associated vasculitis. We also do kidney function tests, including blood and urine tests. The urine test is extremely accurate for picking up any kidney involvement. Blood tests also are done to check for anemia and for markers of inflammation in the blood. These are just the minimum first steps. I really want to run a battery of tests, but at the same time, I really want to make sure that they get treatment right away. Sometimes, we keep a patient in the clinic until we have the results of their kidney urine analysis or we put them in the hospital right away. Other important tests include imaging of the lungs and sinuses. This is done with a CT scan. We also sometimes test to see if there’s nerve involvement, and we may ask our ophthalmologists and ENT [ear, nose, and throat] doctors to look at the sinuses and the eyes.
How do you treat AAV?
There are two goals. The first is to control the disease. We do this in two stages. First is what we call induction therapy. You give patients a lot of medication up front in order to calm down the immune system and induce remission, in which the disease becomes inactive and doesn’t cause symptoms. Induction typically involves high doses of glucocorticoids, which are steroids like prednisone, as well as rituximab (Riabni, Rituxan, Ruxience, Truxima), a biologic drug that suppresses the immune system. Once in remission, we move to the maintenance stage, in which we use lower doses of rituximab or other drugs to keep patients in remission.
The second goal of treatment is to mitigate — as much as possible — the side effects of these medications. Prednisone is a very good medication for suppressing inflammation, but it’s very toxic if you keep taking it. For example, it affects bone health and can lead to fractures. A new drug, avacopan (Tavneos), approved in 2021, has really been a breakthrough, as it helps us reduce the amount of steroids we use. A side effect of drugs like rituximab is an increased risk of infection, so we often use medications like antibiotics to prevent specific infections such as a lung infection called pneumocystis jiroveci pneumonia (PJP).
Can lifestyle changes and other types of self-care help manage AAV?
Yes, and patients are the most important part of treatment. Educate yourself about the disease and make sure you stick to your monitoring plan with your doctor, which means frequent blood and urine tests. Autoimmune diseases like AAV put your heart health at risk, so we often look at risk factors like high cholesterol and use medications to lower cholesterol to levels even lower than those recommended for the general population. In addition to taking medications, you can help protect your heart health if you reduce stress, quit smoking, and make sure you sleep well and exercise regularly. Also, follow a healthy eating plan, such as the Mediterranean diet, that excludes processed foods and limits animal products in favor of plant-based protein.
What message do you like to share with people who have just been diagnosed with AAV?
It’s a serious disease, but treatments today are much better in controlling inflammation and preventing organ damage. So many patients achieve remission and live full lives with proper care.
