Vasculitis is an inflammation of your blood vessels. It causes changes in the walls of blood vessels, including thickening, weakening, narrowing and scarring. These changes restrict blood flow, resulting in organ and tissue damage.
There are many types of vasculitis, and most of them are rare. Vasculitis might affect just one organ, such as your skin, or it may involve several. The condition can be short term (acute) or long lasting (chronic).
Vasculitis can affect anyone, though some types are more common among certain groups. Depending on the type you have, you may improve without treatment. Or you will need medications to control the inflammation and prevent flare-ups.
Vasculitis is also known as angiitis and arteritis.
The signs and symptoms of vasculitis vary greatly and are often related to decreased blood flow throughout the body.
General signs and symptoms common to most vasculitis types
General signs and symptoms of vasculitis include:
- Weight loss
- General aches and pains
- Night sweats
- Nerve problems, such as numbness or weakness
- Loss of a pulse in a limb
Signs and symptoms for some types of vasculitis
Other signs and symptoms are related to specific types of vasculitis. The symptoms can develop early and rapidly or in later stages of the disease.
- Behcet's (beh-CHET'S) syndrome. This condition causes inflammation of your arteries and veins. It generally 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. This condition causes inflammation and clots in the blood vessels of your hands and feet. It can cause pain in your hands, arms, feet and legs, and ulcers on your fingers and toes. This disorder is linked with cigarette smoking. It is also called thromboangiitis (throm-boe-an-jee-I-tis) obliterans.
- Cryoglobulinemia. This condition results from abnormal proteins in the blood. It is often associated with hepatitis C infections. Signs and symptoms include rash, joint pain, weakness, and numbness or tingling.
- Eosinophilic granulomatosis with polyangiitis (also called Churg-Strauss syndrome). This condition is very rare. It mainly affects the kidneys, lungs and nerves in your limbs. Symptoms vary greatly and include asthma, nerve pain and sinus changes.
Giant cell arteritis. This condition is an inflammation of the arteries in your head, especially at the temples. It usually occurs in people over age 50. Giant cell arteritis can cause headaches, scalp tenderness, jaw pain, blurred or double vision, and even blindness. It is also called temporal arteritis.
Giant cell arteritis is linked to polymyalgia rheumatica, which causes pain and stiffness in muscles of the neck, shoulders, hips and thighs.
- Granulomatosis with polyangiitis (also called Wegener's granulomatosis). This condition causes inflammation of the blood vessels in your nose, sinuses, throat, lungs and kidneys. Signs and symptoms include nasal stuffiness, sinus infections and nosebleeds. The affected tissues develop lumps called granulomas. If the lungs are affected, you may cough up blood. The kidneys are often affected. But most people don't have noticeable symptoms until the damage is more advanced.
- Henoch-Schonlein purpura. This condition is an inflammation of the smallest blood vessels (capillaries) of your skin, joints, bowel and kidneys. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs. Henoch-Schonlein usually affects children, but it can occur at any age.
- Hypersensitivity vasculitis. The primary sign of this condition is red spots on your skin, usually on your lower legs. It can be triggered by an infection or an adverse reaction to medicine.
- Kawasaki disease. This condition most often affects children younger than age 5. Signs and symptoms include fever, rash and eye inflammation. It is also called mucocutaneous lymph node syndrome.
- Microscopic polyangiitis. This form of vasculitis affects small blood vessels, usually those in the kidneys and lungs. You may develop abdominal pain and a rash. If the lungs are affected, you may cough up blood.
- Polyarteritis nodosa. This form of vasculitis usually affects the kidneys, the digestive tract, the nerves and the skin. It is often associated with hepatitis B infections. Signs and symptoms include a rash, muscle and joint pain, abdominal pain, high blood pressure, and kidney problems.
- Takayasu's (tah-kah-YAH-sooz) arteritis. This form of vasculitis affects the larger arteries in the body, including the aorta. It typically occurs in young women. Signs and symptoms include a feeling of numbness or cold in the limbs, loss of pulse, high blood pressure, headaches, and visual changes.
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 get worse quickly, so early diagnosis is key to getting effective treatment.
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
Blood vessels affected by vasculitis may bleed or become inflamed. Inflammation 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 and organs.
Complications of vasculitis 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 it. 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). You may need surgery to remove an aneurysm.
- Vision loss or blindness. This is a possible complication if giant cell arteritis is left untreated.
- Infections. These include serious and life-threatening conditions, such as pneumonia and blood infection (sepsis).
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 muscle 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 connective tissue doctors (rheumatologists)
- Brain and nervous system doctors (neurologists)
- Eye doctors (ophthalmologists)
- Heart doctors (cardiologists)
- Infectious diseases doctors
- 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 of ground to cover, 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.
- 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?
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, such as the following:
- Blood tests. These tests can be used to 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. A blood test that looks for certain antibodies — the anti-neutrophil cytoplasmic antibodies test — may be helpful in diagnosing vasculitis.
- Urine tests. These tests may reveal whether your urine contains red blood cells or has too much protein, which can signal a medical problem.
- Imaging tests. Noninvasive imaging techniques can help determine what 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 (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. 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.
Your treatment will be focused on controlling the inflammation with medications and resolving any underlying disease that triggered your vasculitis. For your vasculitis, you may go through two treatment phases — stopping the inflammation and preventing relapse (maintenance therapy).
Both phases involve prescription drugs. Which drugs and how long you need to take them depend on the type of vasculitis, the organs involved and how serious your condition is.
Some people have initial success with treatment, then experience flare-ups later. Others may never see their vasculitis completely go away and need ongoing treatment.
Medications used to treat vasculitis include:
- Corticosteroids to control inflammation. Your doctor may prescribe a corticosteroid drug, such as prednisone or methylprednisolone (Medrol). These help control inflammation in the affected blood vessels. Side effects of corticosteroids can be severe, especially if you take them for a long time. Possible side effects include weight gain, diabetes and bone thinning (osteoporosis). If a corticosteroid is needed for long-term (maintenance) therapy, you'll likely receive the lowest dose possible.
Medications to affect the immune system. If you don't respond to corticosteroids, your doctor may prescribe cytotoxic or immunosuppressant drugs. These types of drugs kill or decrease the function of immune system cells causing the inflammation. They include azathioprine (Azasan, Imuran), methotrexate (Trexall, Rheumatrex) and cyclophosphamide. Possible side effects of cyclophosphamide include increased risk of cancer, infertility and infection. So even though this drug is effective in controlling inflammation, it isn't always the first choice, especially for long-term therapy.
Rituximab (Rituxan) is a safe and effective option for treating some types of vasculitis. Rituximab has proved to be a good option for maintenance therapy, unless you have had hepatitis B. A side effect of rituximab is an increased risk of reactivating hepatitis B.
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 flu and pneumonia, can help prevent problems that can result from your medications, such as infection.
- 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.
Oct. 08, 2014
- What is vasculitis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/vas/. Accessed July 16, 2014.
- Longo DL, et al. Harrison's Online. 18th ed. New York, N.Y.: The McGraw-Hill Companies; 2012. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=4. Accessed July 16, 2014.
- Gwathmey KG, et al. Vasculitic neuropathies. Lancet Neurology. 2014;13:67.
- Guillevin L. Infections in vasculitis. Best Practice & Research in Clinical Rheumatology. 2013;27:19.
- Merkel PA. Overview of the management of vasculitis in adults. http://www.uptodate.com/home. Accessed July 17, 2014.
- Tarzi R, et al. Small vessel vasculitides. Medicine. 2014;42:138.
- Ferri FF. Ferri's Clinical Advisor 2015: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2015. https://www.clinicalkey.com. Accessed July 17, 2014.
- Zand L, et al. Treatment of ANCA-associated vasculitis: New therapies and a look at old entities. Advances in Chronic Kidney Disease. 2014;21:182.
- Murphy JG, et al., eds. Mayo Clinic Cardiology: Concise Textbook. 4th ed. New York, N.Y.: Oxford University Press; 2013.
- Pipitone N, et al. Role of imaging in the diagnosis of large and medium‐sized vessel vasculitis. Rheumatic Disease Clinics of North America. 2013;39:593.
- Warrington KJ, et al. A primer on vasculitis. Minnesota Medicine. 2013;96:36.
- Weyand CM, et al. Giant-cell arteritis and polymyalgia rheumatica. New England Journal of Medicine. 2014;371:50.
- Dammacco F, et al. Therapy for hepatitis C virus-related cryoglobulinemic vasculitis. New England Journal of Medicine. 2013;369:1035.
- Grayson PC, et al. New features of disease after diagnosis in 6 forms of systemic vasculitis. Journal of Rheumatology. 2013;40:1905.
- Specks U, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. New England Journal of Medicine. 2013;369:417.