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

The treatments you receive for AAV will depend on the type you have, and how severe the inflammation is. The treatments for GPA and MPA are usually the same, but EGPA may be treated differently.

Martina (MPA) and Maresa (GPA), Germany

Martina (MPA)
and Maresa
(GPA),
Germany

Non-organ-threatening disease1

Methotrexate
Mycophenolate mofetil
Steroids (glucocorticoids)

Organ- or life-threatening disease1

Cyclophosphamide
Rituximab
Steroids (glucocorticoids)

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, just 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.

A small group of people with organ- or life-threatening disease may also suffer from rapidly progressive renal (kidney) failure or pulmonary haemorrhage. These people may be considered for plasma exchange (cleaning of the blood to remove components that are causing inflammation in AAV).1

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

Signs of renal failure for you to look out for:

 
  • Decreased urine output

  • Swelling in your feet and legs

  • Confusion

  • Fatigue

  • Shortness of breath

Signs of pulmonary haemorrhage for you to look out for:

 
  • Coughing up blood

  • Fever

  • Breathing difficulties

  • A bluish skin tone

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.

How do the different ANCA-associated vasculitis treatments work?

Generally you will be prescribed a combination of therapies to treat your AAV. Each of the therapies act to reduce the activity of the immune system, to stop the inflammation and 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, Ireland

Patricia -
Vasculitis, Ireland

General tips (when receiving treatment for AAV)

Steroids

Rituximab

Cyclophosphamide

Azathioprine

Methotrexate

Mycophenolate mofetil



As AAV treatments affect the immune system and can therefore make you more likely to experience infections, it is advisable to do what you can to minimise your chances of getting sick. The following simple tips are designed to help your body be in the best condition possible when receiving treatment.

  • Wash your hands regularly with soap or antibacterial gel

  • Avoid contact with people who have infections

  • Store and cook food properly

  • Try to keep your house tidy and hygienic, in particular in the kitchen and bathroom

  • If you smoke, try to stop (your doctor will be able to tell you if any services to help you stop smoking are available in your area)

  • Try not to drink alcohol. If you continue to drink, it is important not to have more than 14 units of alcohol per week (the equivalent of 6 pints of beer or 7 glasses of wine), and spread consumption throughout the week

  • Discuss with your doctor what vaccines are suitable for you to help minimise your chances of getting other illnesses.

Why are ongoing clinic visits needed?

Hermann - GPA, Germany

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.

 

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.

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 However, if it is more convenient, you may also be able to arrange for your blood tests to be done separately, with your local doctor.

 

If you have a relapse, your medical team will usually decide whether to manage it by repeating induction therapy or increasing your steroid dose, depending on the severity of relapse.1




Actively monitoring your AAV

Actively monitoring your AAV between visits can help you better understand your disease and spot the signs of any possible relapses as quickly as possible. Some people do this by keeping a diary to track their symptoms. If you decide to keep a diary, you can use it 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 and lifestyle, as well as any other health issues that seem unrelated to the disease.

You can also use your diary to write out questions for your doctor along with anything else that you would like to discuss with them at your next appointment.

If your doctor recommends it, you may also wish to actively monitor your AAV by checking your blood pressure regularly (such as once a week) using a blood pressure monitor and get in touch with your doctors if you notice ongoing changes.

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 lessen the impact of AAV and 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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This Action Plan can help you to stay organised and manage your daily AAV self-care needs. 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, Rheumatologist, Vasculitis patient, artist – 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, Rheumatologist, Vasculitis patient, artist – UK

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General tips (when receiving treatment for AAV)

As AAV treatments affect the immune system and can therefore make you more likely to experience infections, it is advisable to do what you can to minimise your chances of getting sick. The following simple tips are designed to help your body be in the best condition possible when receiving treatment.

  • Wash your hands regularly with soap or antibacterial gel

  • Avoid contact with people who have infections

  • Store and cook food properly

  • Try to keep your house tidy and hygienic, in particular in the kitchen and bathroom

  • If you smoke, try to stop (your doctor will be able to tell you if any services to help you stop smoking are available in your area)

  • Try not to drink alcohol. If you continue to drink, it is important not to have more than 14 units of alcohol per week (the equivalent of 6 pints of beer or 7 glasses of wine), and spread consumption throughout the week

  • Discuss with your doctor what vaccines are suitable for you to help minimise your chances of getting other illnesses.

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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. They reduce inflammation and decrease the activity of the immune system through a few different mechanisms. 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.

Tips when taking steroids

  • Take the tablet in the morning to help you sleep at night

  • Take with breakfast to minimise any stomach upset

  • If you find that steroids cause stomach acid, ask your doctor about whether medicines could help reduce this

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Rituximab

Rituximab is used to treat a variety of autoimmune diseases and certain kinds of cancer. It works to lower the activity of the immune system by affecting the cells which make antibodies. It is normally used in AAV when the disease is active, and is given as an infusion once a week for a month. The infusion usually takes around half a day.

Rituximab can also be used as maintenance therapy (designed to minimise the chances of a relapse) with an infusion every 6 to 12 months. Maintenance therapy is typically done at an outpatient unit or an IV infusion centre.

Tips when taking rituximab

  • It will take 3–5 hours to be given rituximab, so set aside enough time during your day

  • Take some snacks so that you do not become hungry

  • Bring an activity, such as a book to read, to keep you entertained

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Cyclophosphamide

Cyclophosphamide is used to treat various kidney diseases, inflammatory disorders 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 are likely to be less severe.

Cyclophosphamide works by affecting the number and function of white blood cells. It is given as either an infusion every few weeks for around 3 months or a daily tablet. The infusion typically takes several hours. People receiving a course of cyclophosphamide will normally be switched to azathioprine or occasionally mycophenolate mofetil when their course is complete.

Tips when taking cyclophosphamide

  • Drink plenty of fluids (2–3 litres) for 24 hours after the infusion and go to the toilet regularly, as soon as you feel the need to go

  • Take your tablets in the morning on an empty stomach

  • Avoid grapefruit and grapefruit juice, as they can reduce the effectiveness of cyclophosphamide

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Azathioprine

Azathioprine works to reduce the damage and effects caused by a wide range of conditions including Crohn’s disease, rheumatoid arthitis and various autoimmune diseases.

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Methotrexate

Methotrexate is widely used as an anti-inflammatory drug in bone and joint diseases.

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Mycophenolate mofetil

Mycophenolate mofetil helps to reduce the activity of the immune system by affecting white cells, and can be used to treat autoimmune diseases, kidney diseases and transplantation.

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