Treatment of Vasculitis

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.

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.

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.