ANCA vasculitis, or antineutrophil cytoplasmic antibody-associated vasculitis, is the name of a group of autoimmune conditions characterized by the inflammation of the blood vessels caused by the immune system mistakenly attacking them.

There is currently no cure for ANCA vasculitis, but there are treatments available to manage the condition. Current treatments aim to push the condition into remission, where no symptoms appear for a time, and to maintain remission. Depending on the severity and type of ANCA vasculitis, different therapies may be used.

Here is a brief summary of some commonly used treatments.

Glucocorticoids

Glucocorticoids, such as prednisolone, act as an anti-inflammatory. By reducing inflammation, they can reduce the damage caused by the immune system. These are the most commonly used treatments for ANCA vasculitis and are often used in combination with other therapies to induce and maintain remission.

Cyclophosphamide

Cytoxan (cyclophosphamide) was originally approved as a cancer therapy to block cell growth. In ANCA vasculitis, it is believed to kill the immune cells (such as neutrophils) that cause the damaging inflammation. It has been shown to be effective at inducing remission in 90 percent of ANCA vasculitis patients, but can cause severe side effects especially if used long-term.

One of the side effects of cyclophosphamide is hemorrhagic cystitis, a sudden onset of blood in the urine combined with bladder pain. A drug called Mesnex (mesna) can be prescribed to prevent bladder damage if this occurs.

Once remission is achieved, patients are usually switched to a different medication to maintain remission and avoid side effects.

Rituxan

Rituxan (rituximab) is an antibody or protein designed to block a specific target. By inhibiting a protein called CD20, Rituxan reduces the numbers of B-cells. B-cells are a type of immune cells that are involved in producing ANCA, the trigger for the immune system to start attacking the blood vessels.

Rituxan tends to cause fewer severe side effects than Cytoxan. It is now frequently used to induce and maintain remission in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two types of ANCA vasculitis.

Plasma exchange

Plasma is the fluid portion of the blood, which contains the cells (such as red and white blood cells) and other substances. In ANCA vasculitis, it contains the protein ANCA, which triggers the damaging immune response.

Plasma exchange, or PEX, involves taking blood from the patient, separating the plasma, and returning the blood cells to the patient in a substitute fluid.

PEX is frequently used to treat ANCA vasculitis patients, but the exact benefit is unknown. A large scale trial (NCT00987389) called PEXIVAS is currently assessing the effect of PEX in combination with corticosteroids.

Maintenance therapies

Maintainance therapies generally use an immunosuppressant, a drug which reduces the immune response. Commonly used maintenance therapies include azathioprine and methotrexate. If the patient is intolerant or not responding to these treatments, other options include Arava (leflunomide) and CellCept (mycophenolate mofetil). These are often used in combination with glucocorticoids.

Azathioprine, especially, is used as a maintenance therapy for remission in eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome.

Methotrexate or CellCept may be used in combination to induce remission in cases of mild non-organ-threatening ANCA vasculitis.

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