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

AAV Patients - Martina (MPA) and Maresa (GPA), Germany

Martina (MPA)
and Maresa
(GPA),
Germany

Treatments for GPA/MPA

Induction of remission1–4

Steroids
Rituximab
Cyclophosphamide
Methotrexate
Mycophenolate mofetil
Avacopan
Plasma exchange

Maintenance of remission1–3

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

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

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).

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

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

Explore more tips on feeling
in control of AAV

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

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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

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Steroids

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 steroids2,3

  • 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

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Rituximab

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

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Cyclophosphamide

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

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Methotrexate (off-label use)

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

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Mycophenolate mofetil (off-label use)

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

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Avacopan

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

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Plasma exchange

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

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Azathioprine (off-label use)

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

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