Vasculitis

Vasculitis is an inflammation of your blood vessels. Vasculitis causes changes in the walls of your blood vessels, including thickening, weakening, narrowing and scarring. Vasculitis is also called angiitis and arteritis. Many types of vasculitis exist.

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What is it?

Vasculitis is an inflammation of your blood vessels. Vasculitis causes changes in the walls of your blood vessels, including thickening, weakening, narrowing and scarring. Vasculitis is also called angiitis and arteritis. Many types of vasculitis exist.

Vasculitis 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.

Vasculitis can affect anyone, though some types of vasculitis are more common among certain groups. Some forms of vasculitis improve on their own, but others require treatment — often including taking medications for an extended period of time.

Symptoms

General signs and symptoms common to most vasculitis types

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:

  • Fever
  • Fatigue
  • Weight loss
  • Muscle and joint pain
  • Loss of appetite
  • Nerve problems, such as numbness or weakness

Signs and symptoms by type of vasculitis

  • 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 extremities. 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 allergic angiitis, most commonly affects the blood vessels in your lungs. It's often associated with asthma.
  • Cryoglobulinemia. This condition is often associated with hepatitis C infections. Signs and symptoms include a rash called purpura on your lower extremities, arthritis, weakness and nerve damage (neuropathy).
  • Giant cell arteritis. This condition, which occurs in people older than 50, is an inflammation of the arteries in your head, especially your temples. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain while chewing, blurred or double vision, and even blindness. Giant cell arteritis is often associated with polymyalgia rheumatica.
  • Henoch-Schonlein purpura. This condition 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, joint pain, and a rash called purpura on your buttocks, legs and feet. Henoch-Schonlein most often occurs in children, but it can occur at any age.
  • Hypersensitivity vasculitis. The primary sign of hypersensitivity vasculitis is red spots on 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 skin lesions, fever, unintentional weight loss, glomerulonephritis — inflammation of the small blood vessels in the kidneys — and nerve damage.
  • Polyarteritis nodosa. This form of vasculitis affects 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 a rash called purpura, skin ulcers, muscle and joint pain, abdominal pain, and kidney problems.
  • Polymyalgia rheumatica. 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. Polymyalgia rheumatica 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. Many different parts of the body, including the eyes, skin, hands and feet may be involved.
  • 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 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, throat, lungs and kidneys. Signs and symptoms can include shortness of breath, nasal stuffiness, chronic sinusitis, nosebleeds and frequent ear infections.

Causes

Vasculitis occurs when your body attacks your blood vessels. It's not always clear why this happens, but an infection, some cancers, certain immune system disorders or an allergic reaction may serve as the trigger.

Blood vessels affected by vasculitis 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.

Vasculitis with no known cause (primary vasculitis)

For many of its forms, the cause of vasculitis is unknown. These forms of vasculitis are called primary vasculitis.

Vasculitis that occurs due to another disease (secondary vasculitis)

Forms of vasculitis for which an underlying disease is the cause are called secondary vasculitis. Examples of causes of secondary vasculitis include:

  • Infections. Some vasculitis occurs in response to an infection. 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.
  • Immune system diseases. Vasculitis can also occur as the result of some diseases of the immune system, such as rheumatoid arthritis, lupus and Sjogren's syndrome.
  • Allergic reactions. Sometimes an allergic reaction to a medication may cause vasculitis.
  • Blood cell cancers. Cancer that affects the blood cells, including leukemia and lymphoma, can cause vasculitis.

Complications

Complications of vasculitis depend on the type of vasculitis you have. In general, complications that can occur include:

  • Organ damage. Some types of vasculitis can be severe, causing damage to major organs.
  • Recurring episodes of vasculitis. Even when treatment for vasculitis is initially successful, the condition may recur and require further treatment. In other cases, vasculitis may never completely go away and requires ongoing treatment.

Diagnosis

To diagnose vasculitis, your doctor will ask about your symptoms and past medical history and conduct a thorough physical exam. Tests and procedures used to diagnose vasculitis include:

  • Blood tests. Blood tests used to help diagnose vasculitis include erythrocyte sedimentation rate, C-reactive protein test, complete blood cell count and anti-neutrophil cytoplasmic antibodies test.
  • Urine tests. Testing samples of your urine may reveal abnormalities, such as red blood cells and increased amounts of protein, that often indicate a medical problem. If vasculitis has caused kidney problems, your prognosis tends to be poorer.
  • Imaging tests. Your doctor may be able to determine whether larger arteries, such as the aorta and its branches, are affected through the use of noninvasive imaging techniques. These include X-ray, ultrasound, computerized tomography (CT) and magnetic resonance imaging (MRI).
  • X-rays of your blood vessels (angiogram). During an angiogram, a flexible 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.
  • Removing a piece of the affected blood vessel for testing (biopsy). A surgical procedure to remove a small sample (biopsy) of the affected blood vessel allows your doctor to examine the vessel for signs of vasculitis.

Treatment & Management Strategies

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