Central Nervous System Vasculitis
What is vasculitis?
Vasculitis is the inflammation (swelling) of the blood vessels, the network of hollow tubes that carry blood throughout the body. Vasculitis can affect very small blood vessels (capillaries), medium-size blood vessels (arterioles and venules), or large blood vessels (arteries and veins). If blood flow in a vessel with vasculitis is reduced or stopped, the parts of the body that receive blood from that vessel begin to die.
What causes vasculitis?
In most cases, the exact cause is unknown, but the immune system (which helps keep the body healthy) plays a role. While the immune system usually works to protect the body, it can sometimes become "overactive" and attack the body. In most cases of vasculitis, something causes an immune or "allergic" reaction in the blood vessel walls.
Substances that cause allergic reactions are called antigens. Sometimes certain medicines or illnesses can act as antigens and start this process.
What are the symptoms of vasculitis?
Symptoms of vasculitis include:
- skin rashes
- fatigue (tiredness)
- joint pains
- abdominal (stomach) pain
- kidney problems (including dark or bloody urine)
- nerve problems (including numbness, weakness, and pain)
Other symptoms can occur, depending on the area of the body affected by vasculitis. If a blood vessel with vasculitis is small, the vessel may break and produce tiny areas of bleeding in the body. These areas will appear as small red or purple dots on the skin. If a larger vessel is inflamed, it may swell and produce a nodule (lump or mass of tissue), which may be felt if the blood vessel is close to the skin surface.
What is central nervous system vasculitis?
Central nervous system (CNS) vasculitis is inflammation of blood vessel walls in the brain or spine. (The brain and the spine make up the central nervous system.) CNS vasculitis often occurs in the following situations:
- accompanied by other autoimmune diseases such as systemic lupus erythematosus, dermatomyositis, and, rarely, rheumatoid arthritis;
- infection, such as viral or bacterial;
- systemic (affecting the whole body) vasculitic disorders (Wegener’s granulomatosis, microscopic polyangiitis, Behçet’s syndrome);
- it can occur without any associated systemic disorder. In this case, the vasculitis is only confined to the brain or the spinal cord and it is referred to as primary angiitis of the CNS (PACNS).
What is the cause of central nervous system vasculitis?
How the vessels in the brain become inflamed is not entirely clear. In some vasculitic diseases, abnormal antibodies (autoantibodies) attack white blood cells, which attack vessel walls and cause inflammation and destruction of the vessel wall. Infection caused by a virus can also cause CNS vasculitis.
Is central nervous system vasculitis dangerous?
CNS vasculitis can be a serious condition. The inflamed vessel wall can block the flow of oxygen to the brain, causing a loss of brain function. In some cases, CNS vasculitis is life-threatening.
What are the symptoms of central nervous system vasculitis?
Symptoms of CNS vasculitis can include the following:
- severe headaches that last a long time
- strokes or transient ischemic attacks ("mini-strokes")
- forgetfulness or confusion
- problems with eyesight
- encephalopathy (swelling of the brain)
- sensation abnormalities
How is vasculitis diagnosed?
The diagnosis of vasculitis, including CNS vasculitis, is based on a person's medical history, symptoms, a complete physical examination, and the results of special laboratory tests. Blood abnormalities that are found in vasculitis include:
- anemia (a shortage of red blood cells)
- a high white blood cell count
- a high platelet count
- kidney or liver problems
- allergic reactions
- immune complexes
- antibodies (tools the body uses to fight off threats)
- elevation of inflammatory markers
In PACNS, when the vasculitis is only confined to the brain or spinal cord, the above symptoms and signs are often lacking and patients present with symptoms of CNS vasculitis only.
Other tests may include X-rays, tissue biopsies (taking a sample of tissue to study under a microscope), and blood vessel scans. The physician might also want to examine the spinal fluid to see what is causing the inflammation. This test is often performed in CNS vasculitis.
To help in the diagnosis of CNS vasculitis, the physician may order a magnetic resonance imaging or angiogram of the brain. An angiogram can show which blood vessels are narrowed.
Because other conditions can cause some of the same brain vessel abnormalities as CNS vasculitis, a brain biopsy is the only way to make certain of a diagnosis. A brain biopsy can distinguish between CNS vasculitis and other diseases that may have similar features.
How is central nervous system vasculitis treated?
CNS vasculitis is usually treated with steroids. High-dose steroids such as prednisone, in combination with cyclophosphamide (a medication that decreases the immune system’s response to autoimmune diseases), are generally used. In some cases, high-dose steroids alone are tried first; if that does not treat the disease, cyclophosphamide is added. Treatment must be continued for a prolonged period, sometimes for life.
If the patient has another illness (such as lupus) that is related to the vasculitis, then that illness also needs to be treated.
- National Heart, Lung, and Blood Institute. What is vasculitis? www.nhlbi.nih.gov/. Accessed January 11, 2012.
- National Institute of Neurological Disorders and Stroke. Vasculitis Syndromes of the Central and Peripheral Nervous Systems Fact Sheet. www.ninds.nih.gov/. Accessed January 11, 2012.
- Hajj-Ali RA, Singhal A, Benseler S, Molloy E, Calabrese LH. Update on Primary Angiitis of the Central Nervous System. Lancet Neurol 2011 Jun;10(6):561-72.
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