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

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

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

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

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

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