While a diagnosis of antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis, or AAV, may seem daunting, it’s important to know that this chronic autoimmune disorder is no longer the devastating disease it once was. There are many reasons to feel hopeful. “In the beginning, it might feel like, ‘Is my life ever going to go back to normal?’” says Michael Cammarata, MD, a rheumatologist and assistant professor of medicine at Johns Hopkins in Baltimore. “But I think we can confidently reassure our patients that it will.”
AAV causes inflammation of the blood vessels throughout the body. There are three main types: granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. The disease can affect multiple organs — including the kidneys, lungs, sinuses, and skin — but modern treatments have transformed AAV from a once-fatal condition into a manageable chronic illness.
1. Treatment Outcomes Are Much Better Than in the Past
One of the most noteworthy aspects of living with AAV today is that outcomes have improved significantly over the past few decades. “Back in the day, before we had good therapy, [granulomatosis with polyangiitis] was highly fatal from pulmonary and renal failure,” says Dr. Cammarata. “But we have really good therapy now, so we can keep the disease in remission.”
Traditional therapies relied heavily on steroids, and the introduction of rituximab, a synthetic antibody, has been particularly transformative. “Rituximab has led to a complete paradigm shift in the way we manage vasculitis,” says Cammarata. Most people start on rituximab infusions every six months for a few years before decreasing to annual or as-needed infusions (e.g., every few months) based on blood work.
More recently, the addition of avacopan, a twice-daily pill, has allowed doctors to get people with AAV off steroids earlier. “Patients used to be on steroids for months … [or even] indefinitely. But with avacopan up front, we can actually get people off steroids safely, very quickly,” says Cammarata. Long-term steroid use can have serious side effects, including bone loss, increased infection risk, mood changes, and weight gain. And one review article found that avacopan was more effective than the traditional steroid prednisone for sustaining remission at 52 weeks.
2. Most People Achieve and Maintain Remission
The goal of AAV treatment is remission, and the good news is that most people achieve it. Although the disease is prone to relapse, modern medications, combined with regular monitoring, are highly effective at keeping it in check.
Cammarata uses a helpful analogy to explain the treatment approach. “That first phase of disease — when it’s most important to define the extent of the disease and determine which organs are involved — is called ‘remission induction,’ where we’re ‘putting out the fire,’” he says. Once the fire is out, says Cammarata, the ‘remission maintenance’ phase is much simpler. “It’s akin to throwing an occasional bucket of water on the coals to prevent any embers from reigniting,” he says.
Once you’re in remission, life becomes remarkably normal, with follow-up visits that may be spaced out to every three to six months. The goal is to get people back to feeling like their normal selves and living a happy and healthy life without worrying too much about what medications they’re on and any possible side effects, says Cammarata. “Oftentimes in clinics, we see folks who are in remission and doing well,” he says. Many people return to work, maintain relationships, travel, exercise, and pursue the activities they enjoyed before diagnosis.
3. Regular Monitoring May Prevent Serious Complications
While complications such as damage to the kidneys, heart, or nerves can be part of living with AAV, regular monitoring allows doctors to catch problems early, often before you even feel symptoms. “Usually we monitor labs and urine studies every three months,” says Cammarata. “This includes testing blood counts, kidney function, inflammatory markers, and urine protein levels to make sure there’s no brewing disease activity.”
Some people may also need periodic imaging, such as X-rays or CT scans, particularly if they’ve had lung nodules or sinus disease. This proactive approach means that any signs of disease activity can be addressed quickly, preventing the kind of serious complications that were common before modern treatments became available. Your rheumatologist may also monitor your immune system’s response to medications to ensure they’re working effectively.
4. Kidney Function Can Recover
If AAV has affected your kidneys, there’s also reason for hope. Research shows that even people who require dialysis at the time of AAV diagnosis have a chance of kidney recovery. In one review, people with severe kidney involvement who needed dialysis had an over 50 percent chance of discontinuing dialysis after AAV treatment. Meanwhile, the avacopan review found that the drug promoted greater and continued improvement in kidney function.
This underscores the importance of early diagnosis and aggressive treatment. The sooner AAV is identified and treated, the better the chances of preserving or recovering organ function.
5. Research and New Treatments Continue to Advance
The landscape of AAV treatment continues to evolve, with ongoing research aimed at improving long-term outcomes, reducing side effects, and enhancing quality of life.
Studies are examining biomarkers that could help predict relapse risk and guide personalized treatments. This precision medicine approach could allow doctors to tailor therapy to each person, potentially reducing disease activity and medication side effects.
“There are a lot of different CAR T trials ongoing, mostly for lupus and myositis, but I know there are some trying to recruit patients with vasculitis, as well,” says Cammarata. “Whether or not that would be a future treatment strategy for ANCA-associated vasculitis is to be determined, but we’re always looking for medications that will keep patients in durable remission with the fewest — or really no — side effects.”
6. Your Doctors Want You Involved in Treatment Decisions
AAV is complex, and you should feel empowered to ask questions. “Even for doctors and medical students, ANCA-associated vasculitis is a very complicated topic,” says Cammarata. “If you get a diagnosis of one of these diseases, and something about the treatment plan isn’t clear, make that note to your doctor. And make sure that they are explaining things in a way that makes sense.”
Don’t hesitate to speak up if you’re confused or concerned. Understanding why your doctors are making certain recommendations can help reduce anxiety and empower you to be an active participant in your own care.
The Takeaway
- Modern treatments have transformed AAV from a once-fatal disease into a manageable chronic condition.
- The overwhelming early phase of diagnosis and treatment is temporary. Once the disease is controlled, monitoring becomes straightforward, and many people return to full, active lives.
- New trials are exploring treatments such as precision medicine using biomarkers and CAR T-cell therapy, which may effectively treat AAV with fewer side effects.
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