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What is AAV?
Diagnosis
Treatment & self-care
Living with AAV
Carers
Glossary
AAV MASTERCLASS

Glossary

What is AAV?
Diagnosis
Treatment & self-care
Living with AAV
Carers
AAV MASTERCLASS

Available treatments
How do the different treatments work?
Ongoing clinic visits
My self-care needs
Goal setting

What treatments are available for ANCA-associated vasculitis (AAV)?

The treatments you receive for AAV will depend on the type of AAV you have, the disease phase and how severe the inflammation is. The treatments for GPA (Granulomatosis with Polyangiitis, previously called Wegener’s) and MPA (Microscopic Polyangiitis) are usually the same, but EGPA (Eosinophilic Granulomatosis with Polyangiitis, previously called Churg-Strauss syndrome) may be treated differently.¹

Martina (MPA) and Maresa (GPA), Germany

Martina (MPA)
and Maresa
(GPA),
Germany

Treatments for GPA/MPA

Induction of remission^1–4

Steroids

Rituximab

Cyclophosphamide

Methotrexate

Mycophenolate mofetil

Avacopan

Plasma exchange

Maintenance of remission^1–3

Steroids

Rituximab

Azathioprine

Methotrexate

Avacopan*

*Avacopan is recommended for the use in the induction of remission (active disease), and your physician may also decide to use it during the remission of maintenance.¹

The initial goal of treatment is to achieve remission to get the disease under control by using a combination of therapies. This is known as induction of remission. When you achieve remission, you will enter in a new phase called maintenance of remission and the therapies may change.¹

During the induction of remission, either in newly diagnosed patients or in patients who have experienced a relapse, the combination and dose of therapies will depend on whether your disease is organ- or life-threatening or not.¹ While it may be alarming if you are told that your disease is organ- or life-threatening, it is important to be aware that these terms are used to ensure that your doctor is able to give you the best possible care.

For example, having blood in your urine and a slight worsening of kidney function will mean that your disease is medically classed as organ-threatening. However, if these symptoms are successfully managed, your disease will be brought under control and into remission.

Patients with kidney involvement may suffer from rapidly progressive renal (kidney) failure. These people may be considered for plasma exchange (cleaning of the blood to remove components that are causing inflammation in AAV).^1,5

As AAV is a variable disease, you may not be in remission forever. Every person with AAV has a different experience, but if you do relapse, you may need to restart induction treatment.¹

References and footnotes

What does remission mean in AAV?

Remission in AAV means there is no detectable inflammatory disease activity. The term 'remission' implies that the disease is not cured, and you could experience relapses in the future.⁶

Remission is defined as the absence of typical signs, symptoms or other features of active AAV with or without immunosuppressive therapy¹
Sustained remission is defined as the absence of typical signs, symptoms or other features of active AAV over a defined time period with or without immunosuppressive therapy¹

It is important to remember that despite being in remission, it doesn't mean you will necessarily feel perfectly well. You may still require treatment and experience ongoing symptoms, particularly pain and fatigue.^6,7 This is because symptoms can be caused by active disease, organ damage due to the disease or side effects from treatment. For example, even if you are in complete remission, you may still experience symptoms due to accrued organ damage or medication side effects.⁶

Signs of renal failure for you to look out for:⁸

Decreased urine output
Swelling in your feet and legs
Confusion
Fatigue
Shortness of breath

If you are experiencing any of the above symptoms, it is important to contact your doctor immediately so they can examine you and give you the most appropriate treatment.

Lungs - drawn by S. Perera, Artist, Rheumatologist, Vasculitis patient

How do the different ANCA-associated vasculitis treatments work?

Generally you will be prescribed a combination of therapies to treat your AAV. These therapies act to reduce the uncontrolled inflammation and organ damage associated with AAV.¹

Although treatments can help you to manage symptoms, they can also cause side effects and, in some cases, affect your immune system’s ability to fight bacteria and germs,¹ which means you may be more likely to get sick.

If you are experiencing side effects from your medication, it is important to speak to your doctors to see what can be done to help you manage these.

Patricia - Vasculitis patient, Ireland

Patricia -
Vasculitis, Ireland

General tips (when receiving treatment for AAV)

Steroids

Rituximab

Cyclophosphamide

Methotrexate (off-label use)

Mycophenolate mofetil (off-label use)

Avacopan

Plasma exchange

Azathioprine (off-label use)

AAV treatments can have many side effects, so here are some tips designed to help your body be in the best condition possible when receiving treatment:^2–4

Minimise possible situations where infections may be more common, like nurseries, hospitals and airplanes. When you need to attend appointments in clinics or hospitals ensure you protect yourself with a face mask and disinfectant
Try to decrease stress in your life by finding ways to relieve stress, like meditation or yoga
If you smoke, try to stop (your doctor will be able to inform you if any services are available in your area)
Make sure to keep within sensible alcohol limits. This would be no more than 3-4 units of alcohol per day for men, and no more than 2-3 for women. One or two days a week should be alcohol-free
Make sure you stay up-to-date on your vaccinations, and discuss with your doctor what vaccines are suitable for you

The steroids used to treat AAV are called glucocorticoids. They are given in a high dose at first and then gradually reduced if all is going well.¹ They reduce inflammation and decrease the activity of the immune system through a few different mechanisms.³ You may receive steroids throughout your AAV treatment journey, including when you are in remission (the dose is lowered when the disease is not active) and during any periods of relapse.¹

Steroids may be given as tablets or injections (only given by healthcare professionals).^3,5

Tips when taking steroids^2,3

Take tablets in the morning with breakfast to reduce indigestion, heartburn and sleeping difficulties
To reduce the risk of developing osteoporosis, eat foods containing calcium and vitamin D, regularly exercise and take bone strengthening drugs (your doctor should prescribe these for you)
To maintain a healthy weight, keep up your activity levels and adopt a healthy diet

Rituximab is used to treat a variety of autoimmune disease and certain kinds of cancer. It works to lower the activity of the immune system by affecting the cells which make antibodies.⁶

Rituximab is used when the disease is active, and as maintenance treatment when the disease is in remission.¹

Tips when taking rituximab⁷

Your first infusion usually takes 4 to 6 hours so you should plan accordingly
Take activities - infusions last several hours so take something to help pass the time, like a book
Plan for your needs - for example, take a jumper to help you stay warm in case the room is chilly

Cyclophosphamide is used to treat a number of autoimmune conditions and cancers in different doses. However, the doses given to people with AAV will typically be lower than the ones given to treat cancer, so the side effects will be less severe.^8,9

Cyclophosphamide works as an immunosuppressant, decreasing the function of various white blood cells. It is given as an infusion, injection or daily tablet.^8,9 Once patients complete their course of cyclophosphamide or move onto maintenance treatment, they will usually be switched to rituximab (or occasionally methotrexate or azathioprine).¹

Tips when taking cyclophosphamide⁸

Take cyclophosphamide in the morning
Prior, during or immediately after taking, drink plenty of fluids and go to the toilet regularly
Avoid grapefruit and grapefruit juice, as they can reduce the effectiveness of cyclophosphamide

Methotrexate is an immunosuppressant, often used to treat different cancers, neoplastic diseases and psoriasis.¹⁰
Methotrexate is not clinically indicated for use in GPA/MPA, but the EULAR 2022 recommendations suggest it can be used as an alternative to rituximab in remission induction and maintenance treatment.^1,10

Mycophenolate mofetil reduces the activity of the immune system by targeting white blood cells.¹¹ Mycophenolate mofetil is not clinically indicated for use in GPA/MPA, but the EULAR 2022 recommendations suggest it can be used as an alternative to rituximab in remission induction treatment.^1,11

In AAV, a specific protein in the body called a C5a receptor plays a key role in stimulating inflammation.¹² Avacopan attaches to this receptor and prevents it from working, thereby reducing inflammation of blood vessels seen in these diseases.¹³

Avacopan is a prescription medicine used to treat adults with severe, active GPA or MPA. It is used together with other treatments prescribed by your doctor known as rituximab or cyclophosphamide.¹³

Things you should know when taking Avacopan¹³

Avacopan needs to be taken twice a day, in the morning and evening with food. It may help to create a routine, for example taking it with breakfast and dinner
Grapefruit and grapefruit juice are to be avoided as this can influence the effect of avacopan
Inform your doctor whenever you start taking a new medicine

Plasma exchange is a procedure where your blood is cleaned to remove the ANCAs and other inflammatory triggers.¹² Plasma exchange is only recommended for consideration in patients with rapidly progressive glomerulonephritis (kidney inflammation).¹

Tips when receiving plasma exchange¹⁴

Drink plenty of fluids before the session
Wear comfortable clothing
Bring a book or something else to entertain you during the procedure

Azathioprine is an immunosuppressant, used to treat many autoimmune conditions and in cases of organ transplant.¹⁵ Azathioprine is not clinically indicated for use in GPA/MPA, but the EULAR 2022 recommendations suggest it can be used as an alternative to rituximab in maintenance treatment.^1,15

References and footnotes

Why are ongoing clinic visits needed?

Ongoing clinic visits allow your doctors to check that your disease is under control and monitor you for any signs of relapse or new organ involvement.¹

Urine testing is typically performed at each visit to screen for infection, kidney problems or bladder complications. Every 1–3 months, your inflammatory markers and renal (kidney) function will be measured to monitor your disease.¹

You will also have blood tests to monitor blood count and liver function so your doctors can see how your medication is affecting your body.¹ Patients on glucocorticoid therapy should also have their blood glucose measured periodically.¹

If you have a relapse, you will usually have to repeat induction therapy again.²

Actively monitoring your AAV

Actively monitoring your AAV between visits can help you better understand your disease and can help your spot the signs of any possible relapses. Some people do this by keeping a diary to track their symptoms. By keeping a diary this will help you to discuss your AAV with your doctors.

Hermann - GPA, Germany

You can write in your diary each day or just use it when your circumstances change. Topics that may be useful to include are your symptoms, treatment, side effects, and AAV's impact on your everyday life.

Use your diary to write out questions for your doctor.

If your doctor recommends it, you may also wish to actively monitor you AAV by checking your blood pressure regularly (such as once a week).

References and footnotes

How can I manage my self-care needs?

It can be difficult to manage your daily AAV self-care needs and challenges. Staying organised and on top of these tasks can help improve your overall health and wellness.¹

Self-care can involve requests from your doctor, as well as steps you need to take to manage your AAV between
visits.^2–4

References and footnotes

Use the Action Plan checklist below to help reduce the impact of AAV on your everyday life:¹

Take medicines as prescribed by your doctor Try to take steps to avoid infection (such as washing hands) and get vaccinated when needed Keep up with all doctor visits and health-related exams Track and report progress with your doctor, such as positive changes, symptoms and treatment side-effects Report any serious concerns to your doctor promptly Try to eat healthy foods to support overall wellness Stay active and try to maintain valued roles (parenting, work, hobbies) Adjust your routine when needed, and consider what you can do to manage symptoms, treatment, and other self-care needs

Explore more tips on feeling
in control of AAV

Explore more

References and footnotes

How can goal setting help?

Many people with AAV find that setting goals helps them become a more active participant in their disease management by giving them short- or long-term targets to aim for.

Shanali, Artist, Rheumatologist, Vasculitis patient - UK

Realistic goal setting

Realistic goal setting is important to avoid disappointment or frustration, and your doctors will be able to discuss which goals might be suitable for you, based on factors such as your disease, treatment, and personal, social or professional ambitions.

Shanali, Artist, Rheumatologist, Vasculitis patient - UK

1. Hellmich B, et al. Ann Rheum Dis 2023;0:1–18.

2. TAVNEOS EU SmPC July 2023.

3. MabThera 100 mg Concentrate for Solution for Infusion SmPC October 2021.

4. Cyclophosphamide 500 mg Powder for Solution for Injection or Infusion SmPC April 2021.

5. Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017;12(10):1680-91.

6. Vasculitis Foundation (2012). What is remission? Available at: https://www.vasculitisfoundation.org/mcm_faq/what-is-remission/. Last accessed: June 2023.

7. ANCA Vasculitis News (2022). AAV Patients Report Pain, Fatigue Despite Being in Clinical Remission. Available at: https://ancavasculitisnews.com/news/aav-patients-report-pain-fatigue-despite-being-clinical-remission/. Last accessed: June 2023.

8. Mayo Clinic (2022). Acute kidney failure. Available at: https://www.mayoclinic.org/diseases-conditions/kidney-failure/symptoms-causes/syc-20369048. Last accessed: June 2023.

1. Yates M, et al. Ann Rheum Dis 2016;75(9):1583–94.

2. Hellmich B, et al. Ann Rheum Dis 2023;0:1–18.

1. Clark NM, et al. J Aging Health 1991;3:3–27.

2. Gallant MP. Health Educ Behav 2003;30:170–95.

3. Thorpe TT, et al. Arthritis Rheum 2007;57(7):1296–302.

4. Yates M, et al. Ann Rheum Dis 2016;75(9):1583–94.

1. Thorpe TT, et al. Arthritis Rheum 2007;57(7):1296–302.

1. Hellmich B, et al. Ann Rheum Dis 2023;0:1–18.

2. The Lauren Currie Twilight Foundation (2013). Eating Well with Vasculitis. Available at: https://www.thelaurencurrietwilightfoundation.org/downloads/Fact_Sheets/Guidance/Vasculitis-Diet.pdf. Date accessed: November 2022.

3. NHS (2020). Steroid Tablets. Available at: https://www.nhs.uk/conditions/steroid-tablets/. Date accessed: June 2023.

4. IVX Health (2014). How to Stay Healthy While Taking Immunosuppressive Meds. Available at: https://ivxhealth.com/blog/how-to-stay-healthy-while-taking-immunosuppressive-meds/. Last accessed: June 2023.

5. NHS (2023). Steroid injection. Available at: https://www.nhs.uk/conditions/steroid-injections/. Last accessed: June 2023.

6. MabThera 100 mg Concentrate for Solution for Infusion SmPC October 2021.

7. Rituxan. Preparing for your RITUXAN infusion. Available at: https://www.rituxan.com/ra/treatment/preparing.html#:~:text=PREPARING%20FOR%20YOUR%20RITUXAN%20INFUSION&text=Your%20first%20infusion%20usually%20takes,or%20bring%20a%20snack%20along. Last accessed: June 2023.

8. Cyclophosphamide 500 mg Powder for Solution for Injection or Infusion SmPC April 2021.

9. Cyclophosphamide Tablets 50mg SmPC December 2016.

10. Methotrexate 2.5mg Tablets SmPC January 2023.

11. Cellcept 500mg Film-Coated Tablets SmPC July 2022.

12. Jennette JC, Nachman PH. Clin J Am Soc Nephrol 2017;12(10):1680-91.

13. TAVNEOS EU SmPC July 2023.

14. Healthline (2018). Plasmapheresis: What to expect. Available at: https://www.healthline.com/health/plasmapheresis. Last accessed: June 2023.

15. Azathioprine Tablets 25mg SmPC August 2022.

General tips (when receiving treatment for AAV)

AAV treatments can have many side effects, so here are some tips designed to help your body be in the best condition possible when receiving treatment:^2–4

Minimise possible situations where infections may be more common, like nurseries, hospitals and airplanes. When you need to attend appointments in clinics or hospitals ensure you protect yourself with a face mask and disinfectant
Try to decrease stress in your life by finding ways to relieve stress, like meditation or yoga
If you smoke, try to stop (your doctor will be able to inform you if any services are available in your area)
Make sure to keep within sensible alcohol limits. This would be no more than 3-4 units of alcohol per day for men, and no more than 2-3 for women. One or two days a week should be alcohol-free
Make sure you stay up-to-date on your vaccinations, and discuss with your doctor what vaccines are suitable for you

Steroids