Questions And Answers
General
What exactly is vasculitis and what are the different types?
The term ‘vasculitis’ is used to describe inflammation of the blood vessels. This may occur in large or small vessels, therefore various organs of the body can be affected, such as the kidneys, lungs, nerves, joints, eyes, brain, etc. It is therefore said to be a multisystem or systemic disease.
Main types of vasculitis are:
- Giant Cell (Temporal) Arteritis
- Takayasu's Arteritis
- Polyarteritis Nodosa
- Granulomatosis with Polyangiitis (formerly Wegener's Granulomatosis)
- Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome)
- Microscopic Polyangiitis
Each type of vasculitis affects different blood vessels and organs, leading to varying symptoms and complications. Common features of all these diseases are that the inflamed blood vessels allow less blood to reach the organs. This can cause damage to the organ itself, which can be progressive and sometimes permanent.
How many cases of vasculitis are there in Europe/worldwide, in total?
Vasculitis is a relatively rare disease but it is estimated that approximately 300,000 people in Europe are living with vasculitis.
How many new cases in Europe are there each year?
In Europe, approx 15,000 new patients a year are diagnosed with vasculitis, so approximately 13-30 new cases per million per year.
Are cases on the increase?
Data on vasculitis shows an increase in diagnosed cases, however whether this is due to a real increase in the disease, or simply due to better diagnostic awareness or other factors, is unclear.
Is vasculitis more common in certain groups?
We know that autoimmune disorders in general occur more commonly in women. Depending on the type of vasculitis, we have seen some trends, e.g.:
- Giant cell arteritis is more common in women are than men and patients are usually over 50 years of age
- Granulomatosis with polyangiitis (GPA) is more common in northern Europe, whereas microscopic polyangiitis is more commonly seen in east Asia and southern parts of Europe.
Are there specialist clinics for vasculitis? How do I access these?
Yes, there are centres of excellence set up worldwide for various diseases, and it is especially important to be seen or referred to one if you have a rare disease, as you can access more specialist care. Your healthcare team can put you in touch with these.
How is vasculitis treated? What are the side effects?
Treatment will depend on the type of vasculitis you have and on your symptoms. Broadly speaking, the goal of treatment is to control inflammation, relieve symptoms, prevent organ damage, and maintain remission. Treatment may involve the use of medications such as corticosteroids, immunosuppressants, or biologic agents, and that long-term management is typically necessary. Immunosuppressive drugs are used to achieve this but these drugs have their own side effects. Usually, the patient is given a large immunosuppressive dose initially, followed by smaller maintenance doses, to try to control the inflammation. But this approach needs to be customized to the individual, since vasculitis can flare up at certain times, it’s not predictable. This is a big challenge for doctors.
Early diagnosis, prompt treatment, and adherence to medical advice are the keys to managing the condition effectively.
Is vasculitis hereditary?
No genetic cause has been identified for vasculitis, therefore it is not considered to be a hereditary disorder. However, it is possible for a family to share certain versions of genes which predispose them to autoimmune diseases, which may include vasculitis.
- Does vasculitis reduce lifespan?
- How many people with vasculitis die each year?
- Does it cause blindness?
- How long does it take skin to return to normal? Does it leave scars?
- Is vasculitis progressive? If skin and bones are affected, will it eventually spread to other organs?
Data on vasculitis – do we know:
What research is being conducted currently?
Research teams around the world are currently studying patient data, clinical test results, biomarkers, genetic screening, etc and trying to link it all together to create predictive tests for flares or ways to improve treatments.
This research can help us be able to predict which patients really need immunosuppressive drugs, and spare those that don’t.
What we’ve learned so far:
- PR3-ANCA antibody seems to be associated with more frequent relapse
-
A urine protein test, which can be performed in clinic, can show kidney vasculitis flares, indicate kidney damage and can save you having a kidney biopsy.
Attacks and flare-ups
- Are there any known factors that bring on a flare-up (e.g., sun, food, footwear, injuries, etc) and should be avoided? Is there a way to reduce risk of episodes?
- Does vasculitis always return or can you have one severe episode and it never comes back? Relapse can be very variable, again depending on the type of vasculitis you have. The majority of vasculitis patients are likely to relapse at some stage, but remission is possible through treatment. It is important to notice any new or persistent symptoms you may be experiencing, and to report these to your healthcare team in a timely manner. Unfortunately, we do not currently have a specific test or marker to predict flares but this is something that is being researched. The sooner flares can be treated, the better - the less damage will be done, the less medication dose you may need.
- Do flare ups often attack the same system again or is that also variable? The symptoms you see in flare-ups are really just the tip of the iceberg about what’s going on in the body, and for how long. So things can vary in relapse, though typically a person with kidney involvement will show kidney symptoms again.
- What percentage of people never have another attack and what percentage have recurring attacks? Does it flare up during pregnancy or affect childbirth, or fertility etc?
- What over the counter medicines are good to take during a flare up?
Resources and advice
Are there any tips from sufferers for coping with different symptoms and their experience of living with these?
Receiving a vasculitis diagnosis can be a terribly difficult time, because you’re told it’s a rare disease and you can feel quite isolated and uncertain of the future. It is important to have regular medical check-ups, follow treatment plans, manage your symptoms, and seek support from healthcare professionals, family, and support groups.
It’s important to get good information from trusted sources, listen to your body and be very open with your healthcare team, and give them feedback. Quality of life is very important, so it’s important to tell your team about the small stuff as well as the big things that are affecting you.
It can be helpful to bring someone with you to your appointments early on, as you may not be able to take in everything initially, it can be an overwhelming time.
It’s important to ask questions too, and learn as much as you can to manage your illness. Very important that it’s a two-way conversation between patient and physician, since the patients are the ones who are experiencing the disease, whereas the physicians have to rely on test data.
Joining a patient group can be helpful – there are now vasculitis groups and charities around the world which are putting patients in touch. Joining these groups can be daunting but take it at a level that suits you, people are at all different stages themselves so don’t feel like you have to share everything. The groups can offer education and support and it can be good to meet people going through the same thing as you.
Receiving a diagnosis can be life-changing but give yourself time, go easy on yourself. Things will be different than before, you will need to adjust.
Common myths and misconceptions about the condition?
People with vasculitis should stay on top of their vaccination and health screenings, including COVID vaccination. Vaccination is effective in most people with vasculitis, so better to get vaccinated.
There is no data to suggest that vaccination (for COVID or other viruses) precipitates vasculitis.
Vasculitis Question Time Webinar
To celebrate International Vasculitis Day in 2023, a Vasculitis Question Time was hold where a team of specialists from both the Rheumatology and Nephrology fields, answered some frequently asked questions about Vasculitis. This Webinar was organised on the initiave of RITA Patients Representatives.