The treatments used for vasculitis will depend upon which blood vessels and organs are affected, as well as how much body tissue is affected. If the vasculitis only affects the skin, it may be enough to treat any underlying infection or to remove the drug that triggered the vasculitis. However, in most cases, drug treatment will be needed to control the disease and its symptoms and to stop or limit the damage caused by vasculitis.
The two main types of drug used to treat vasculitis are steroids and immunosuppressant agents. Both act to dampen down the immune system to reduce the strength of its attack on the tissues of the body.
If you have vasculitis affecting the large blood vessels, then you'll probably be given steroid tablets. These are very effective for giant cell arteritis and Takayasu arteritis, and often immunosuppressive agents aren't needed for these conditions.
If you have vasculitis affecting small and/or medium-sized blood vessels, then you may only need a small dose of steroids to control it.
However, you may need a combination of steroids and immunosuppressive drugs, probably over several years, especially if vasculitis affects internal organs. For many types of vasculitis, including those affecting the kidney, lungs or other vital organs (especially if it involves both small and medium-sized blood vessels), your treatment will be given in stages.
If you have vasculitis that mainly affects medium-sized arteries, then other treatments can help, depending on the condition:
- Kawasaki disease can be treated effectively with the injections of immunoglobulin (a type of protein).
- Hepatitis-associated PAN can be treated with antiviral treatment and plasma exchange.
Plasma exchange (also known as blood washing) involves being connected to a machine that your blood passes through before being returned to you so it can be cleaned of the factors causing the vasculitis. Only a few people with the most severe types of vasculitis – for example who have very severe kidney or lung disease – will need plasma exchange. This will be done in specialist centres.
Possible side-effects of steroids include weight gain, indigestion, diabetes, thinning of the skin and thinning of the bones (osteoporosis). If high doses of steroids are given, then you'll also be given drugs like bisphosphonates to help prevent osteoporosis.
The stages of treatment for some types of vasculitis:
Stage 1: Bringing you to remission. The aim is to get the disease under control. An immunosuppressive drug (for example cyclophosphamide) may be used to dampen down the immune system, which is attacking the blood vessels. Steroid tablets or injections will also be used in this stage.
Stage 2 Keeping you in remissions. Once the condition is in remission, the aim is to keep it under control. Drugs (for example azathioprine, methotrexate or mycophenolate) may be used with steroids.
Stage 3: Following up and withdrawing therapy. The aim at this stage is to gradually reduce your medications while still keeping the disease under control.
In some types of vasculitis (such as granulomatosis with polyangiitis), an infection may trigger a relapse. You may therefore be given antibacterial drugs such as co-trimoxazole to protect against this. These drugs can also help to protect against the increased risk of infection caused by the stronger immunosuppressive drugs.
As with all medications, there may sometimes be side-effects.
Cyclophosphamide, for example, can cause bleeding from the bladder, hair thinning and an increased risk of infection. Unfortunately, there's also a significant risk that it can reduce fertility in both men and women.
Because of these risks, cyclophosphamide will be stopped or exchanged for a different immunosuppressive drug as soon as your vasculitis is controlled. This is usually azathioprine, but methotrexate or mycopheonlate might be used instead. Rituximab, a biological therapy given by intravenous infusion, can also help to encourage remission in some types of vasculitis.