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Vasculitis, also known as angiitis and arteritis, is the inflammation of blood vessels. This causes the blood vessels to thicken, weaken and narrow, which decreases the flow of blood and therefore oxygen and vital nutrients. Vasculitis damages the body’s tissues and organs.

Although there are many types of vasculitis, most of them are rare. The disease can be acute or chronic and affect one or several organs. Anyone can experience this condition, however some kinds of vasculitis are much more common among specific groups of people. Depending on the type of vasculitis you are diagnosed with, you may get better with no treatment. Some types require medical treatment.

Symptoms

General symptoms of vasculitis include fever, fatigue, muscle and joint pain, numbness and weight loss. Some are only associated with a specific type of vasculitis. Symptoms can begin at the onset of the illness and progress rapidly or develop over time.

Specific Types of Vasculitis

  • Behcet’s (beh-CHETS) disease– Behcet’s disease causes swelling in your veins and arteries. You may develop ulcers in the mouth and genital area. Other symptoms include eye inflammation and lesions on the skin that are similar to acne.
  • Cryoglobulinemia – This type of vasculitis occurs due to abnormal proteins in the blood and can cause joint pain, weakness, tingling, numbness and rash.
  • Giant cell arteritis – Also known as temporal arteritis, giant cell arteritis causes the arteries in your head to swell, mainly at the temples. Symptoms include headaches, jaw pain, double or blurry vision, scalp tenderness, and blindness.
  • Churg-Strauss syndrome (Eosinophilic granulomatosis with polyangiitis) – This type of vasculitis typically affects the skin, lungs, heart, kidneys and the nerves in the arm or legs. It is very rare. If you have this type of vasculitis, you may experience nerve pain, allergic rhinitis (nasal allergies), skin changes, or asthma.
  • Henoch-Schonlein purpura (IgA vasculitis) – More common in children, this condition causes the capillaries in the skin, kidneys, bowels and joints to swell. Symptoms include joint and abdominal pain and blood in the urine. It also causes a rash to develop on the lower legs and backside.
  • Granulomatosis with polyangiitis – This type of vasculitis causes the blood vessels in the nose, throat, lungs, sinuses and kidneys to swell. Common signs of this condition include nosebleeds, sinus infections and potentially coughing up blood. However, many people do not experience any symptoms until the illness progresses.
  • Hypersensitivity vasculitis – Also known allergic vasculitis, this condition occurs due to infection and as a result of an adverse allergic reaction to medication. Common signs include red spots on the skin, typically on the lower part of your legs.
  • Kawasaki disease – Kids younger than 5-years-old are more likely to develop this type of vasculitis, which is also known as mucocutaneous lymph node syndrome. Symptoms include eye redness, rash and fever.
  • Microscopic polyangiitis – Typically this condition only affects the small blood vessels in the lungs, nerves and kidneys. You may cough up blood if your lungs are affected. Other symptoms include weight loss, muscle pain, fever, as well as pain in the abdominal area.
  • Takayasu's (tah-kah-YAH-sooz) arteritis - Takayasu's arteritis commonly affects the larger arteries of the body, as well as the aorta. If you experience this condition, you may have joint pain, high blood pressure, fever, loss of appetite, vision problems, headaches and overall discomfort.
  • Polyarteritis nodosa – Generally, polyarteritis nodosa only affects the skin, nerves, digestive system and the kidneys. Common symptoms include high blood pressure, pain in the abdomen after eating, weight loss, a rash, joints and muscle pain, kidney issues and overall discomfort.

What causes vasculitis?

The exact cause of vasculitis has not been determined. In some cases genetics plays a role. Vasculitis can also develop due to an abnormal reaction of the immune system as a result of infections, blood cancers and autoimmune disorders, like rheumatoid arthritis and systemic lupus. Vasculitis may also develop as a reaction to specific drugs.

Complications

The complications associated with vasculitis depends upon the type and severity of the condition. Also, problems may occur as a side effect of a prescribed medicine used to treat vasculitis. Complications include:

  • Blood clots and aneurysms – A blood clot can develop within a blood vessel, which prevents blood flow. Also, an affected blood vessel can bulge or weaken to form an aneurysm. This is very rare.
  • Damage to organs – Specific organs in the body can become significantly damaged. This depends on the type of vasculitis and the severity.
  • Infection – Some types of vasculitis can cause serious infection, including sepsis (blood infection) and pneumonia.

How is vasculitis diagnosed?

Your physician will complete a comprehensive physical exam and discuss your medical history. You may receive one or more diagnostic tests and procedures, including:

  • Urine test –This test identifies the amount of protein in your blood and the presence of red blood cells. Red blood cells and too much protein in the urine can indicate a medical issue.
  • Blood test – Your doctor may recommend a complete blood cell count to assess whether or not you have enough red blood cells and to identify the amount of C-reactive protein in your blood. A large amount of C-reactive protein is a sign of inflammation. A blood test can also indicate the presence of specific antibodies in your blood.
  • Biopsy – Your doctor will remove a small sample of tissue from the affected area to check for signs of vasculitis.
  • Imaging tests – An X-ray, ultrasound, CT scan or MRI can help your physician diagnose vasculitis and also track your treatment progress.
  • X-ray of the blood vessels – During this diagnostic test, your physician will insert a catheter into a large artery or vein. A special contrast dye is injected into the catheter. While the dye fills the vein or artery, X-rays are taken, giving your doctor a good view of the blood vessels.
  • What treatment options are available?

    Your treatment plan will consist of two phases – stopping the inflammation and preventing reoccurrence. In some cases, treatment is initially successful, but flare-ups may occur in the future. If your vasculitis does not go away completely, you will need ongoing medical treatment.

    What types of medications are used to treat vasculitis?

    Treatment options include corticosteroids, such as prednisone or methylprednisolone (medrol). These medicines are quite effective, but can cause serious side effects if they are taken long-term. Common side effects include diabetes, weight gain and osteoporosis. If you need to take a corticosteroid long-term, your doctor will prescribe the lowest dosage possible.

    In some cases, your physician may prescribe steroid-sparing drugs like methotrexate or azathioprine (Imuran), along with a corticosteroid, so that you can be taken off the corticosteroid quicker. Biologic therapy drugs, such as rituximab (Rituxan) may be recommended. The medications you will have to take depends on the kind of vasculitis you have, the affected organs, and any other health problems you are experiencing.

    Surgery

    If an aneurysm (bulge) forms in the wall of a blood vessel, you may need surgery. You may also need surgery to treat a blocked artery.

    If not treated, vasculitis can be serious and cause permanent nerve and organ damage. Tissues and organs can become damaged if the blood vessels become inflamed and narrowed, limiting the amount of blood to those areas.

    Common tests to diagnose vasculitis include blood tests, physical exam, X-rays, biopsies and angiography.

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