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Vasculitis

Definition:
Vasculitis is an inflammation of your blood vessels, which include your veins, arteries and capillaries. Also called angiitis, vasculitis causes changes in the walls of your blood vessels, such as 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 vasculitis can affect people of all ages. Some age groups are affected more than others, depending on the type of vasculitis. However, all types of vasculitis are rare. Though some forms of vasculitis improve on their own, others require treatment — often including taking medications for an extended period of time.

Causes :
Your vascular system is an intricate network of blood vessels, including veins, arteries and capillaries. If all of your blood vessels were laid end to end, they would extend the length of nearly 60,000 miles. 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.

For many forms of vasculitis, the cause 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, and the hepatitis B virus 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.

When to seek medical advice:
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.

Symptoms:
The signs and symptoms of vasculitis vary depending on which vessels and, as a result, which organ systems are affected. However, general signs and symptoms that most people with vasculitis experience include:
  • Fever
  • Fatigue
  • Weight loss
  • Muscle and joint pain
  • Loss of appetite
Each type of vasculitis can also cause specific signs and symptoms, such as:
  • Behcet's syndrome. This condition causes inflammation of your arteries and veins, and often appears in your 20s and 30s. Signs and symptoms include mouth and genital ulcers, eye inflammation and acne-like lesions on your skin.
  • Buerger's disease. Also called thromboangiitis obliterans, this condition causes inflammation and clots in the blood vessels in your arms and legs. Signs and symptoms can include pain in your hands, arms, feet and legs, and ulcers on your fingers and toes. This disorder is strongly associated with cigarette smoking.
  • Churg-Strauss syndrome. This condition, also known as allergic granulomatosis and angiitis, most commonly affects the blood vessels in your lungs. It's often associated with asthma, allergies and an increased number of a specific type of white blood cells in the blood (eosinophilia).
  • Cryoglobulinemia. This condition is often associated with hepatitis C infections. Signs and symptoms include a hemorrhagic rash (purpura) on your lower extremities, arthritis, weakness and nerve damage (neuropathy).
  • Giant cell arteritis (GCA). This condition, which usually occurs in people older than 50, is an inflammation of the arteries in your neck, upper body, arms, and — most often — head, especially the temples. GCA is the most common type of vasculitis in the United States and can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. GCA is often associated with polymyalgia rheumatica and stiffness and aching in the neck, shoulder and hip girdle regions.
  • Henoch-Schonlein purpura. This condition, previously called allergic or anaphylactoid purpura, is caused by inflammation of the blood vessels of your skin, joints, bowel and kidneys. Signs and symptoms can include abdominal pain, blood in the urine or stool, joint pain, and a hemorrhagic rash (purpura) on your buttocks, legs and feet. Henoch-Schonlein most often occurs in children, but it can occur at any age.
  • Hypersensitivity vasculitis. This type of vasculitis involves the small blood vessels of your skin. It can be triggered by an allergy, most often to a medication or an infection.
  • Kawasaki disease. Also known as mucocutaneous lymph node syndrome, this condition most often affects children younger than 5 years of age. Signs and symptoms include fever, skin rash and eye inflammation.
  • Microscopic polyangiitis. This form of vasculitis affects small-sized blood vessels in your kidneys, lungs and skin. Signs and symptoms include purpura, glomerulonephritis — inflammation of the small blood vessels in the kidneys — and pulmonary hemorrhage.
  • Polyarteritis nodosa. This form of vasculitis affects small- to medium-sized blood vessels in many different parts of the body, including your skin, heart, kidneys, peripheral nerves, muscles and intestines. Signs and symptoms include purpura, skin ulcers, muscle and joint pain, abdominal pain, and high blood pressure (hypertension).
  • Polymyalgia rheumatica (PMR). This condition primarily affects older adults and results in pain and inflammation of the large joints, such as your shoulders, hips and knees. Signs and symptoms include pain and stiffness in the muscles of your hips, thighs, shoulders, upper arms and neck. PMR often occurs in association with giant cell arteritis.
  • Rheumatoid vasculitis. This type of vasculitis can complicate the course of rheumatoid arthritis and usually occurs in people with a history of severe rheumatoid arthritis.
  • Takayasu's arteritis. This form of vasculitis includes the largest arteries in the body, including the aorta, and typically occurs in young women. Signs and symptoms include back pain, arm weakness or pain with use (claudication), decreased or absent pulses, lightheadedness, headaches, and visual disturbances.
  • Wegener's granulomatosis. This condition causes inflammation of the blood vessels in your nose, sinuses and throat (collectively called the upper respiratory tract), lungs, and kidneys. Signs and symptoms can include shortness of breath, nasal pain and stuffiness, nosebleeds, and ulcers in your nose.

Diagnosis:
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 you having 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 antineutrophil 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.
  • Urine test. This test may detect abnormalities, such as red blood cells and increased amounts of protein, in your urine that often indicate a medical problem. If the kidneys are involved, your prognosis tends to be poorer.
Complications:
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.

Treatment:
Your specific treatment regimen depends on your type of vasculitis, the severity of your case and your general health. Your doctor will ask about your past reactions in regard to medications, nutritional supplements, and sun exposure, which can bring on allergic skin rashes. 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.
  • Cytotoxic drugs. Some cases of vasculitis that are severe or that don't respond well to corticosteroids may need treatment with cytotoxic drugs, such as azathioprine (Imuran) and cyclophosphamide (Cytoxan). These drugs suppress the inflammation in your blood vessels. Mycophenolate mofetil (CellCept), another immunosuppressant used to prevent transplant rejection, has been used to treat vasculitis, though the Food and Drug Administration hasn't approved it for this purpose.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs, such as aspirin and ibuprofen (Advil, Motrin, others), can be effective in treating mild symptoms of some types of vasculitis, such as polymyalgia rheumatica or Kawasaki disease. But NSAIDs don't offer complete relief for many people, and long-term use can cause stomach and intestinal bleeding.
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 the tumor necrosis factor (TNF) blockers (Remicade, Enbrel) and rituximab (Rituxan).
 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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