Vasculitis is an inflammation of your blood vessels. Also called angiitis, vasculitis causes changes in the walls of your blood vessels, including thickening, weakening, narrowing and scarring. Inflammation can be short-term (acute) or long-term (chronic) and can be so severe that the tissues and organs supplied by the affected vessels don't get enough blood. The shortage of blood can result in organ and tissue damage, even death.
There are many types of vasculitis and, although rare, vasculitis can affect anyone. Some age groups are affected more than others, depending on the type of vasculitis. Some forms of vasculitis improve on their own, but others require treatment — often including taking medications for an extended period of time.
The signs and symptoms of vasculitis vary depending on which blood vessels and, as a result, which organ systems are affected. However, general signs and symptoms that most people with vasculitis experience include:
Each type of vasculitis can also cause specific signs and symptoms, such as:
Your vascular system is an intricate network of blood vessels, including veins, arteries and capillaries. Systemic arteries deliver oxygen-rich blood to your body's tissues, while veins return blood with increased amounts of carbon dioxide — a waste product of metabolism — to your heart. Capillaries, the smallest blood vessels, connect the veins and the arteries and permit the transfer of fluids and nutrients to and from the surrounding tissues.
In vasculitis, the blood vessels become inflamed, which can cause the layers of the blood vessel wall to thicken. This narrows the blood vessels, reducing the amount of blood — and therefore oxygen and vital nutrients — that reaches your body's tissues. In some cases, a blood clot may form in an affected blood vessel, obstructing blood flow. Sometimes instead of becoming narrower, a blood vessel may weaken and form a bulge (aneurysm), a potentially life-threatening condition.
Primary vs. secondary
For many of its forms, the cause of vasculitis is unknown. These forms of vasculitis are called primary vasculitides.
For some types, however, infections may be the cause. Forms of vasculitis for which an underlying disease is the cause are called secondary vasculitides. For instance, most cases of cryoglobulinemia are the result of the hepatitis C virus infection, and the hepatitis B virus infection causes some cases of polyarteritis nodosa. Vasculitis can also occur as the result of some diseases of the immune system, such as rheumatoid arthritis, lupus and Sjogren's syndrome. Sometimes an allergic reaction to a medication, such as an antibiotic or diuretic, may cause vasculitis.
See your doctor if you think that you have vasculitis. Some forms of vasculitis can be severe — affecting critical organs — and can lead to death if you don't receive treatment.
If you've already been diagnosed with vasculitis, keep in mind that the signs and symptoms of a disease flare (recurrence) are often similar to those that occurred when the disease first began. In addition, be aware of any new signs or symptoms, as these may indicate either a disease flare or the development of a complication of treatment, such as an infection.
The signs and symptoms of vasculitis resemble those of many conditions, which can make a definite diagnosis difficult. As a result, your doctor will try to rule out other possible causes of your condition.
He or she will ask about your symptoms and past medical history and conduct a thorough physical exam. You may also have some of the following tests:
Blood tests. If your doctor suspects vasculitis, he or she may order a blood test that checks your erythrocyte sedimentation rate — commonly referred to as the sed rate. This test measures how quickly red blood cells fall to the bottom of a tube of blood. Red cells that drop rapidly may indicate inflammation in your body.
You also may have a test that measures C-reactive protein (CRP), a substance produced by your liver in response to inflammation.
Your doctor may also check the number of red blood cells for anemia and platelets (thrombocytes) in your blood. Platelets are colorless blood cells that help stop blood loss when you're injured. Some types of vasculitis result in an unusually high or low number of these cells.
Your doctor may also check to see if you have a high white blood cell count, which can signify an infection or inflammation.
In addition, your doctor may check your blood for anti-neutrophil cytoplasmic antibodies (ANCAs) and other antibodies, such as rheumatoid factor (RF) and antinuclear antibody (ANA). ANCA can indicate a diagnosis of Wegener's granulomatosis or microscopic polyangiitis. RF and ANA elevations can be indications of an associated rheumatoid arthritis or connective tissue disease.
Imaging studies. Your doctor may be able to determine whether larger arteries, such as the aorta and its branches, are involved through the use of noninvasive imaging techniques. These include ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI).
In some cases, though, you may need a more invasive X-ray test called an angiogram. During this procedure, a catheter, resembling a thin straw, is inserted into a large artery or vein. A special dye (contrast medium) is then injected into the catheter, and X-rays are taken as the dye fills these arteries or veins. The outlines of your blood vessels are visible on the resulting X-rays.
Biopsy. Although blood tests and imaging studies can provide your doctor with useful information, one of the best ways to confirm a diagnosis of vasculitis is by taking a small sample (biopsy) of the affected blood vessel. The procedure is performed on an outpatient basis under local anesthesia, usually with very little discomfort or scarring.
The sample is examined for signs of inflammation under a microscope in a laboratory. If there is evidence of scarring, it implies that the condition has been chronic, or long-standing. Treatment may not be as effective in these cases, because the damage has been done and may not be reversible.
Many cases of vasculitis are minor and either run their course without treatment, such as some cases of Henoch-Schonlein purpura, or can be effectively controlled by treatment.
However, some cases of vasculitis are severe and involve major organ systems. In these cases, damage to the organs may occur before treatment has time to work, or the condition may be so severe as to resist treatment. These cases can result in major organ damage or death.
Even when treatment for vasculitis is initially successful, the condition may recur later and require further treatment. Giant cell arteritis, Wegener's granulomatosis and Takayasu's arteritis all are types of vasculitis that often recur after initial remission. In other cases, vasculitis may never completely go away and requires ongoing treatment.
Specific treatment for vasculitis depends on your type of vasculitis, the severity of your case and your general health. Your doctor will ask if you've had any past reactions to medications, nutritional supplements and sun exposure, all of which may bring on allergic skin rashes in some people.
Though some types of vasculitis are self-limiting and improve on their own, such as Henoch-Schonlein purpura, others involve taking one or more of the following medications:
Corticosteroids. Treatment for many types of vasculitis consists of doses of a corticosteroid drug, such as prednisone or methylprednisolone (Medrol). You often start feeling better in just a few days, but you may need to continue taking medication for an extended period of time. After the first month, your doctor may gradually begin to lower the dose until you reach the lowest dose of corticosteroids you need to control inflammation. Some of your signs and symptoms may return during this tapering period.
Your doctor may also have you take medications to combat the side effects of long-term treatment with corticosteroids. For instance, the bisphosphonates risedronate (Actonel) and alendronate (Fosamax) can treat steroid-induced osteoporosis. Risedronate is also approved for the prevention of osteoporosis.
While you undergo treatment, your doctor may conduct periodic blood tests to monitor your progress and response to treatment.
Other medications that researchers are studying in the treatment of vasculitis include mycophenolate mofetil (CellCept), tumor necrosis factor (TNF) blockers (Remicade, Enbrel) and rituximab (Rituxan).
When vasculitis is identified and treated early, the prognosis is usually good. One of your greatest challenges may be coping with any side effects of your medication. The following suggestions may help:
Eat a healthy diet. Eating well can help prevent potential problems that can result from your medications, such as thinning bones, high blood pressure and diabetes. Emphasize fresh fruits and vegetables, whole grains, and lean meats and fish, while limiting salt, sugar and alcohol.
Be sure to get adequate amounts of calcium and vitamin D. If you find it hard to get calcium from your diet because you can't eat dairy products, for example, try calcium supplements, which are often combined with vitamin D. Supplements are effective, inexpensive, and generally well tolerated and well absorbed if taken properly.
Sometimes calcium supplements can cause constipation. If this is a problem for you, drink more water and try using a fiber supplement. In addition, check the type of calcium you're using. Calcium phosphate and calcium citrate tend to be less constipating than other types.
Good food sources of calcium include milk; low-fat plain yogurt; Swiss, cheddar and ricotta cheeses; broccoli; canned salmon with the bones; and orange juice and other products, such as tofu, fortified with calcium.
