Treatment of Vasculitis

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The treatment of choice for most systemic forms of vasculitis is a course of corticosteroids. In vasculitides that require high doses of corticosteroids or if they produce a lot of side effects, then they are often coadministered with an immunosuppressant.

Non-drug therapy

Apple

Diet. Given that systemic vasculitides are inflammatory diseases and they affect the blood vessels, there is an inherent risk that the inflammation itself could accelerate the process of atherosclerosis, which may result in serious mid- or long-term problems. Therefore, patients with vasculitis must focus on healthy lifestyle habits to fully minimise any cardiovascular risk factors, for example by abstaining from smoking and alcohol, and by preventing and treating obesity, high blood pressure, diabetes, high blood cholesterol and hyperuricaemia.

Cigarette crossed out on a "no smoking" poster

Tobacco. Cardiovascular risk factors can be managed with drugs used to control high blood pressure, high cholesterol and uric acid levels and diabetes, but it is vital that patients do not smoke or drink alcohol and follow a healthy diet that is low in fat and sugar and with very little or no salt (to improve blood pressure, fluid retention and address any increased appetite associated with the corticosteroids).

Bottle with correct calcium drink

Calcium. Dairy products should form part of the diet as they supply calcium to prevent or improve osteoporosis.

Man running; regular physical exercise

Exercise. Practising any aerobic exercise, from walking to participating in sports, is highly recommended as it reduces cardiovascular risk and helps retain muscle strength, which is often weakened by both the condition and due to the corticosteroid therapy.

Drug therapy

Round pills

The treatment of choice for almost all systemic vasculitides are corticosteroids, normally at high doses (adjusted to the patient’s weight), administered either orally (prednisone) or intravenously (methylprednisolone). Intravenous administration is normally used in more severe cases and at higher doses. An initial intense, acute treatment (called remission induction therapy) is followed by a more moderate but prolonged regimen in which the dose of corticosteroids is progressively reduced (called remission maintenance).

Hormone therapy pills

In cases of giant cell arteritis (GCA) and mild forms of polyarteritis nodosa (PAN), treatment should start with just corticosteroids alone. On the other hand, for Takayasu’s arteritis (TKA) and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] and eosinophilic granulomatosis with polyangiitis [GEPA]), corticosteroid therapy is usually initiated in conjunction with another immunosuppressant such as methotrexate, azathioprine or cyclophosphamide.

Person lying in bed resting

The most recommended treatment in mild cases of IgA-associated vasculitis (IgAV) is rest and symptomatic management, while corticosteroids and other immunosuppressants are reserved for more severe or extended cases or those with a negative evolution. In Kawasaki’s disease (KD), the primary treatment comprises a course of high-dose aspirin and corticosteroids are subsequently administered if the disease does not develop favourably.

Surgical treatment

Surgical treatment is reserved for patients with occlusive vascular conditions that involve a lack of blood supply or ruptured blood vessels which damage an organ or tissue, or that are potentially life-threatening.

Forms of large-vessel arteritis (giant cell arteritis and Takayasu’s arteritis) can block the main arteries supplying blood to the brain, limbs, kidneys and vital organs. They can also cause dilatations or aneurysms in the aorta, alter the aortic valve and induce heart failure.

Of the medium-vessel vasculitides, polyarteritis nodosa can obstruct, dilate or rupture arteries in the abdomen (intestine, liver, stomach, spleen or kidneys), the brain or any other part of the body, while Kawasaki’s disease usually causes dilatations (aneurysms) in the coronary arteries.

Whenever any of these alterations are severe, they require, to a varying degree of urgency, open surgery or an endovascular procedure (using a catheter). Small-vessel vasculitides do not usually produce complications that can be resolved through surgery as they only have a diffuse effect on organs and regions.

New therapies. Biological agents

Traditional corticosteroids and immunosuppressants produce widespread side effects because they have a generalised action on all cells and the mechanisms regulating the defences that attack the body’s own tissues in the case of inflammation.

To improve the efficacy of the medications and produce less side effects, researchers have studied and developed new substances and drugs designed to act on certain cells or more specific aspects of the autoimmune or inflammatory mechanisms that trigger diseases such as vasculitis. These molecules, which are manufactured synthetically, are called biological agents and include antibodies that act against specific molecules involved in inflammation as well as substances that directly interrupt the inflammatory mechanisms.

The following biological agents have proven effective in certain types of vasculitis:

  • Tumour necrosis factor (TNF) inhibitors such as infliximab, etanercept or adalimumab in the treatment of Takayasu’s arteritis.
  • Anti-interleukin-6 (IL-6) agents such as tocilizumab for giant cell arteritis (GCA) and Takayasu’s arteritis.
  • Anti-CD20 monoclonal antibodies (anti-B-cell) in ANCA-associated vasculitides such as granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and cryoglobulinaemic vasculitis (particularly in association with hepatitis C virus infection).
  • Anti-IL-5 agents such as mepolizumab in the case of eosinophilic granulomatosis with polyangiitis (EGPA).

There are other biological agents that cannot be recommended across the board in these and other types of vasculitis, since they have only been tested in isolated cases or are currently being studied in clinical trials which have not yet yielded any results.

Treatment complications

The long-term use of corticosteroids is associated with side effects such as weight loss, fat accumulation (Cushing’s syndrome), muscle atrophy and the development of osteoporosis, high blood pressure and glaucoma. Furthermore, some traditional immunosuppressants are related with a decreased blood cell count (e.g., leucocytes, red blood cells and platelets) and a minimal risk of developing cancer (above all in patients administered high doses over a long period). Corticosteroids and various immunosuppressants (traditional and biological) share the risk of producing infections because not only do they reduce the body’s defences to prevent it from being attacked by its own immune system, but they also reduce its ability to fight off the germs that cause infections.

Substantiated information by:

Georgina Espigol Frigolé
José Hernández Rodríguez
Maria Cinta Cid Xutgla
Sergio Prieto González

Published: 30 November 2018
Updated: 30 November 2018

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