Vasculitis

Overview

Vasculitis involves inflammation of the blood vessels. The inflammation can cause the walls of the blood vessels to thicken, which reduces the width of the passageway through the vessel. If blood flow is restricted, it can result in organ and tissue damage.

There are many types of vasculitis, and most of them are rare. Vasculitis might affect just one organ, or several. The condition can be short term or long lasting.

Vasculitis can affect anyone, though some types are more common among certain age groups. Depending on the type you have, you may improve without treatment. Most types require medications to control the inflammation and prevent flare-ups.

Symptoms

General signs and symptoms of most types of vasculitis include:

  • Fever
  • Headache
  • Fatigue
  • Weight loss
  • General aches and pains

Other signs and symptoms are related to the parts of the body affected, including:

  • Digestive system. If your stomach or intestines are affected, you may experience pain after eating. Ulcers and perforations are possible and may result in blood in the stool.
  • Ears. Dizziness, ringing in the ears and abrupt hearing loss may occur.
  • Eyes. Vasculitis can make your eyes look red and itch or burn. Giant cell arteritis can cause double vision and temporary or permanent blindness in one or both eyes. This is sometimes the first sign of the disease.
  • Hands or feet. Some types of vasculitis can cause numbness or weakness in a hand or foot. The palms of the hands and soles of the feet might swell or harden.
  • Lungs. You may develop shortness of breath or even cough up blood if vasculitis affects your lungs.
  • Skin. Bleeding under the skin can show up as red spots. Vasculitis can also cause lumps or open sores on your skin.

When to see a doctor

Make an appointment with your doctor if you have any signs or symptoms that worry you. Some types of vasculitis can worsen quickly, so early diagnosis is the key to getting effective treatment.

Causes

The exact cause of vasculitis isn't fully understood. Some types are related to a person's genetic makeup. Others result from the immune system attacking blood vessel cells by mistake. Possible triggers for this immune system reaction include:

  • Infections, such as hepatitis B and hepatitis C
  • Blood cancers
  • Immune system diseases, such as rheumatoid arthritis, lupus and scleroderma
  • Reactions to certain drugs

Risk factors

Vasculitis can happen to anyone. Factors that may increase the risk of certain disorders include:

  • Age. Giant cell arteritis rarely occurs before the age of 50, while Kawasaki disease is most common in children younger than 5 years old.
  • Family history. Behcet's disease, granulomatosis with polyangiitis and Kawasaki disease sometimes run in families.
  • Lifestyle choices. Using cocaine can increase your risk of developing vasculitis. Smoking tobacco, especially if you're a man younger than 45, can increase your risk of Buerger's disease.
  • Medications. Vasculitis can sometimes be triggered by medications such as hydralazine, allopurinol, minocycline and propylthiouracil.
  • Infections. Having hepatitis B or C can increase your risk of vasculitis.
  • Immune disorders. People who have disorders in which their immune systems mistakenly attack their own bodies may be at higher risk of vasculitis. Examples include lupus, rheumatoid arthritis and scleroderma.
  • Sex. Giant cell arteritis is much more common in women, while Buerger's disease is more common in men.

Complications

Vasculitis complications depend on the type and severity of your condition. Or they may be related to side effects of the prescription medications you use to treat the condition. Complications of vasculitis include:

  • Organ damage. Some types of vasculitis can be severe, causing damage to major organs.
  • Blood clots and aneurysms. A blood clot may form in a blood vessel, obstructing blood flow. Rarely, vasculitis will cause a blood vessel to weaken and bulge, forming an aneurysm (AN-yoo-riz-um).
  • Vision loss or blindness. This is a possible complication of untreated giant cell arteritis.
  • Infections. Some of the medications used to treat vasculitis may weaken your immune system. This can make you more prone to infections.

Diagnosis

Your doctor likely will start by taking your medical history and performing a physical exam. He or she may have you undergo one or more diagnostic tests and procedures to either rule out other conditions that mimic vasculitis or diagnose vasculitis. Tests and procedures might include:

  • Blood tests. These tests look for signs of inflammation, such as a high level of C-reactive protein. A complete blood cell count can tell whether you have enough red blood cells. Blood tests that look for certain antibodies — such as the anti-neutrophil cytoplasmic antibody (ANCA) test — can help diagnose vasculitis.
  • Imaging tests. Noninvasive imaging techniques can help determine which blood vessels and organs are affected. They can also help the doctor monitor whether you are responding to treatment. Imaging tests for vasculitis include X-rays, ultrasound, computerized tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET).
  • X-rays of your blood vessels (angiography). During this procedure, a flexible catheter, resembling a thin straw, is inserted into a large artery or vein. A special dye is then injected into the catheter, and X-rays are taken as the dye fills the artery or vein. The outlines of your blood vessels are visible on the resulting X-rays.
  • Biopsy. This is a surgical procedure in which your doctor removes a small sample of tissue from the affected area of your body. Your doctor then examines this tissue for signs of vasculitis.

Treatment

Treatment focuses on controlling the inflammation and managing any underlying conditions that may be triggering the vasculitis.

Medications

A corticosteroid drug, such as prednisone, is the most common type of drug prescribed to control the inflammation associated with vasculitis.

Side effects of corticosteroids can be severe, especially if you take them for a long time. Possible side effects include weight gain, diabetes and weakened bones. If a corticosteroid is needed for long-term therapy, you'll likely receive the lowest dose possible.

Other medications may be prescribed with corticosteroids to control the inflammation so that the dosage of corticosteroids can be tapered more quickly. The medication used depends on the type of vasculitis that is present. These medications may include methotrexate (Trexall), azathioprine (Imuran, Azasan), mycophenolate (CellCept), cyclophosphamide, tocilizumab (Actemra) or rituximab (Rituxan).

The specific medications that you'll need depend on the type and severity of vasculitis you have, which organs are involved, and any other medical problems that you have.

Surgery

Sometimes, vasculitis causes an aneurysm — a bulge or ballooning in the wall of a blood vessel. This bulge may need surgery to reduce the risk of it rupturing. Blocked arteries also may require surgical treatment to restore blood flow to the affected area.

Coping and support

One of your greatest challenges of living with vasculitis may be coping with side effects of your medication. The following suggestions may help:

  • Understand your condition. Learn everything you can about vasculitis and its treatment. Know the possible side effects of the drugs you take, and tell your doctor about any changes in your health.
  • Follow your treatment plan. Your treatment plan may include seeing your doctor regularly, undergoing more tests and checking your blood pressure.
  • Choose 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. Choose a diet that emphasizes fresh fruits and vegetables, whole grains, low-fat dairy products, and lean meats and fish. If you're taking a corticosteroid drug, ask your doctor if you need to take a vitamin D or calcium supplement.
  • Get routine vaccinations. Keeping up to date on vaccinations, such as for the flu and pneumonia, can help prevent problems that can result from your medications, such as infection. Talk to your doctor about vaccinations.
  • Exercise most days of the week. Regular aerobic exercise, such as walking, can help prevent bone loss, high blood pressure and diabetes that can be associated with taking corticosteroids. It also benefits your heart and lungs. In addition, many people find that exercise improves their mood and overall sense of well-being. If you're not used to exercising, start out slowly and build up gradually. Your doctor can help you plan an exercise program that's right for you.
  • Maintain a strong support system. Family and friends can help you as you cope with this condition. If you think it would be helpful to talk with other people who are living with vasculitis, ask a member of your health care team about connecting with a support group.

Preparing for an appointment

Make an appointment with your primary care doctor if you have signs or symptoms that worry you. If your doctor suspects that you have vasculitis, he or she may refer you to a joint and autoimmune disease specialist (rheumatologist) with experience in helping people with this condition. You may also benefit from a multidisciplinary approach. What specialists you see depends on the type and severity of your condition.

Specialists who treat vasculitis include:

  • Joint and autoimmune disease doctors (rheumatologists)
  • Brain and nervous system doctors (neurologists)
  • Eye doctors (ophthalmologists)
  • Heart doctors (cardiologists)
  • Kidney doctors (nephrologists)
  • Lung doctors (pulmonologists)
  • Skin doctors (dermatologists)
  • Urinary and urogenital system doctors (urologists)

What you can do

Because appointments can be brief, and because there's often a lot to discuss, it's a good idea to be well prepared. Try to:

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, ask if you need to do anything in advance, such as restrict your diet.
  • Send previous test information. If you've been referred to a larger medical center, ask your home doctor to forward your previous imaging and biopsy results before the appointment.
  • List any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • List key personal medical information, including other recent health problems or major stresses you've had and any medications, vitamins and supplements you're taking.
  • Consider taking a family member or friend with you to the appointment. Someone who accompanies you can help remember what the doctor says.
  • List questions you want to ask your doctor.

For vasculitis, some basic questions to ask include:

  • What type of vasculitis do I have?
  • What's causing my vasculitis?
  • Will I need more tests?
  • Is my vasculitis acute or chronic?
  • Will my vasculitis go away on its own?
  • Is my vasculitis serious?
  • Has any part of my body been seriously damaged by vasculitis?
  • Can my vasculitis be cured?
  • What are my treatment options?
  • What are the benefits and risks of each treatment?
  • Is there one treatment you feel is best for me?
  • How long will treatment last?
  • I have another medical condition. How can I best manage these conditions together?
  • Should I see a specialist?
  • Do you have any brochures or other printed material that I can take with me? What websites do you recommend?

What to expect from your doctor

Your doctor may ask:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

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