Vasculitis

What is Vasculitis?

Vasculitis is an autoimmune disease in which the body mistakenly sees blood vessels as a foreign invader and attacks them, causing inflammation and leading to a narrowing of the vessels. Vasculitis can occur by itself or can be a feature of a rheumatic disease, such as rheumatoid arthritis, systemic lupus erythematosus (SLE; lupus) or systemic sclerosis. In severe cases, patients can develop organ damage or death. About 100,000 Americans per year are admitted to the hospital because of vasculitis. Vasculitis can affect people of all ages, races and gender.

There are many types of vasculitis. They are classified according to the size of the blood vessels affected.

  • Large vessel. Polymyalgia rheumatic, Takayasu's arteritis, temporal arteritis (and giant cell arteritis)
  • Medium vessel. Buerger's disease, cutaneous vasculitis, Kawasaki disease, polyarteritis nodosa
  • Small vessel. Behçet's syndrome, Churg–Strauss syndrome, cutaneous vasculitis, Henoch–Schönlein purpura, microscopic polyangiitis, Wegener's granulomatosis, Golfer's vasculitis, cryoglobulinemia

Vasculitis Causes

Vasculitis occurs when the immune system attacks the blood vessels by mistake. What causes this to happen isn't fully understood. Sometimes an autoimmune disorder triggers vasculitis. Autoimmune disorders occur when the immune system makes antibodies (proteins) that attack and damage the body's own tissues or cells.

Vasculitis Symptoms

Signs and symptoms of vasculitis vary. They depend on the type of vasculitis, the organs involved and the severity of the condition. Some people may have few signs and symptoms. Other people may become very sick. Sometimes symptoms develop slowly, over months. Other times, the signs and symptoms start quickly, over days or weeks.

Systemic or constitutional symptoms are those that affect the body in a general way. Common systemic symptoms of vasculitis are:

  • Fever
  • Loss of appetite
  • Weight loss
  • Fatigue
  • General aches and pains

Vasculitis can affect specific organs and body systems, causing a range of signs and symptoms.

  • Skin. Purple or red spots or bumps; clusters of small dots, splotches, bruises, or hives; itching
  • Joints. Aching or arthritis in one or more joints
  • Lungs. Shortness of breath; coughing up blood
  • Gastrointestinal tract. Sores in the mouth; stomach pain; in severe cases, blockage of blood flow to the intestines that can cause weakening or rupture of intestines
  • Sinuses, nose, throat, and ears. Sinus or chronic middle ear infections; sores in the nose; in some cases, hearing loss
  • Eyes. Red, itchy, burning eyes; light sensitivity; blurred vision; rarely, blindness
  • Brain. Headaches; problems thinking clearly; changes in mental function; stroke-like symptoms, such as muscle weakness and paralysis
  • Nerves. Numbness, tingling and weakness in various parts of the body; loss of feeling or strength in hands and feet; shooting pains in arms and legs

Vasculitis Diagnosis

Vasculitis is diagnosed based on signs and symptoms, medical history, a physical exam, and test results.

  • Laboratory tests. Blood and urine tests may show whether you have abnormal levels of certain blood cells and antibodies (proteins) in your blood.
  • Biopsy. This is often the best way to make a firm diagnosis of vasculitis. During a biopsy, the doctor takes a small sample of an affected blood vessel or organ to study under a microscope, looking for signs of inflammation or tissue damage.

Other possible tests and examinations the doctor may perform will depend on the symptoms, and may include:

  • Blood pressure measurement
  • Urinalysis
  • Electrocardiogram (EKG)
  • Echocardiography
  • Chest X-ray
  • Lung function tests
  • Abdominal ultrasound
  • Computed tomography (CT) scan
  • Magnetic resonance imaging (MRI)
  • Angiography

Vasculitis Treatment

Treatment for vasculitis depends on the type of vasculitis, which organs are affected and the severity of the condition. The main goal of treating vasculitis is to reduce inflammation in the affected blood vessels. People who have severe vasculitis are treated with prescription medicines. People who have mild vasculitis may find relief with over-the-counter pain medicines, such as acetaminophen, aspirin, ibuprofen or naproxen.

Common prescription medicines used to treat vasculitis include corticosteroids and disease-modifying antirheumatic drugs (DMARDs). Corticosteroids help reduce inflammation in your blood vessels. Examples are prednisone, prednisolone and methylprednisolone. Doctors may prescribe DMARDs if vasculitis is severe or if corticosteroids don't work well. These drugs kill the cells causing the inflammation. Examples are azathioprine, methotrexate and cyclophosphamide.

Vasculitis Self Care

Self management involves understanding and following the treatment prescribed by doctors and other healthcare providers. It also important to make lifestyle choices and address the physical and emotional effects of having a rheumatic or autoimmune disease like vasculitis. Self management encompasses the choices made each day to live well and stay healthy.

If you think you have a type of vasculitis, find out when to see a doctor.

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