Randy Jacobs, M.D. Patient Education

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What is Vasculitis?
Vasculitis (pronounced vaa-skew-lite-iss) is inflammation of the blood vessels. Inflammation is a condition in which tissue is damaged by blood cells which enter it. These are mostly white blood cells which circulate and are our major defense against infection. Ordinarily, they destroy bacteria and viruses. However, these cells can also damage normal tissue if they invade it. The blood vessels which become inflamed in vasculitis can be very small (capillaries), medium-size (arterioles or venules), or large (arteries or veins). Several things can happen to a blood vessel which is inflamed. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue, such as small red or purple dots on the skin. If it is larger, it may swell and produce a nodule (which may be felt if the blood vessels is close to the skin surface). The inside of the vessel tube may become narrowed so that blood flow is reduced, or the inside may become totally closed (usually by a blood clot which forms at the same place there is inflammation). If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die. For example, a person with vasculitis of a medium-sized artery in the hand may develop a cold finger which hurts whenever it is used. Occasionally gangrene sets in.

What Causes Vasculitis?
Vasculitis can be caused by (1) Invasion of blood vessels walls by infectious agents, or (2) An immune or "allergic" reaction going on in the vessel walls. The first cause is not very common. When it occurs, bacteria, viruses or fungi infect the blood vessel, white blood cells move in to destroy the infectious agents and damage the blood vessel during the process. This is a serious illness and requires prompt antibiotic treatment. The second cause of vasculitis, an immune reaction, is more common. Substances which cause allergic reactions are called "antigens." Antigens cause the body to make proteins called "antibodies" which bind to the antigen for the purpose of getting rid of it. A drug allergy can cause this type of reaction. Antigen and antibody bound together are called "immune complexes." Two primary ways in which immune complexes destroy antigens are: (1) By attracting white blood cells to digest the antigen, and (2) By activating other body substances (such as complement) which help in that destruction. For example, hepatitis viruses can be inactivated by both anti hepatitis antibodies and by special white blood cells, produced by the body for the purpose of destroying hepatitis virus. Unfortunately, some immune complexes do not serve their purpose of destroying antigens. They remain too long in the body and circulate in the blood and deposit in tissues. They commonly deposit in blood vessel walls, where they cause inflammation. It is likely that some white blood cells programmed to kill infectious agents ("cyto-toxic") can also accidentally damage blood vessels and cause vasculitis. For example, some unlucky individuals, when infected with the hepatitis virus, develop not only liver inflammation, but also hives, arthritis and vasculitis all caused by immune complexes containing viral antigens, antibodies, and complement, and the anti-viral white blood cells. In the vasculitis caused by lupus, the antigens causing the immune complexes are often not known. In some cases, the complexes can contain DNA and anti-DNA, or the Ro (also called SS-A) antigen and anti-Ro. The complexes can be detected in the blood and levels of complement can be measured. Vasculitis can be seen on biopsies of affected tissues and the immune complexes can be seen with special tests of the biopsy material.

Symptoms of Vasculitis
Vasculitis can cause many different symptoms, depending upon what tissues are involved, and the severity of the tissue damage. Some patients are not ill and notice occasional spots on their skin; others are very ill with systemic symptoms and major organ damage. A list of symptoms based on the tissues in which vasculitis occurs include:

  • Systemic symptoms: Fever, generally feeling bad ("malaise"), diffuse aching in muscle and joints, poor appetite, weight loss and fatigue. This set of complaints are not at all specific to vasculitis.
  • Skin: Red or purple dots, usually most numerous on the legs (these are called "petechiae"). When the spots are larger, about the size of the end of a finger, they are called "purpura". Some look like large bruises. These are the most common vasculitis skin lesions, but hives, itchy lumpy rash, and painful or tender lumps can occur. Areas of dead skin can appear as ulcers (especially around the ankles), small black spots at the ends of the fingers or around the fingernails and toes ("nail fold infarcts"), or gangrene.
  • Joints: Aching in joints and a frank arthritis with pain, swelling and heat in joints. Deformities are rare.
  • Brain: Vasculitis in the brain can cause many problems, from mild to severe. They include headaches, behavioral disturbances, confusion, seizures, and strokes.
  • Peripheral Nerves: Peripheral nerve symptoms may include numbness and tingling (usually in an arm or a leg, or in areas which would be covered by gloves or socks), loss of sensation (especially in the feet and hands), or strength .
  • Intestines Inadequate blood flow in the intestines can cause crampy abdominal pain and bloating. If areas in the wall of the intestine develop gangrene, blood will appear in the stool. If the intestinal wall develops a hole (called a "perforation"), surgery may be required.
  • Heart: Vasculitis in the coronary arteries is unusual in lupus. If it occurs, it can cause a feeling of heaviness in the chest during exertion ("angina"), which is relieved by rest. Frank heart attacks can occur.
  • Lungs: Vasculitis in this tissue can cause pneumonia-like attacks with chest X-ray changes that look like pneumonia and symptoms of fever and cough. Occasionally, inflammation can lead to scarring of lung tissue with shortness of breath.
  • Kidneys: Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis. It may not cause any symptoms, although most patients with renal vasculitis have high blood pressure.
  • Eyes: Vasculitis involving the small blood vessels of the retina can occur in lupus. The retina is a tissue at the back of the eye which contains cells that have to be activated to form a visual image. Sometimes, vasculitis of the eyes causes no symptoms. Usually, however, there is visual blurring which comes on suddenly and stays, or even loss of a portion of the vision. In other non-lupus types of vasculitis, such as temporal arteritis, there is sudden loss of part of the vision in one eye, usually accompanied by headache.

Diseases Associated with Vasculitis
Vasculitis can occur in many different illnesses almost any situation where there are antigen, antibody, and immune white blood cells. Vasculitis can also occur by itself without any obvious infection or other illness. Drug allergy is a common cause . Some of the illnesses associated with vasculitis include:

- Infections - Temporal Arteritis - Scleroderma
- Autoimmune Diseases - Cryoglobulinemia - Leukemia
- Lupus - Erythema Nodosum - Lymphoma
- Rheumatoid Arthritis - Wegener's Granulomatosis - Tumors

Consulting Dr. Jacobs
If you suspect that you or a friend or relative has vasculitis, you should consult Dr. Jacobs as soon as possible. Remember, vasculitis can be very mild and of little importance, or very severe and life-threatening or any degree in between. Therefore, an expert should help to decide: (a) If you have vasculitis, (b) How serious it is and (c) If and how it should be treated. Doctors trained in several different specialties are taught to recognize and treat vasculitis. These include rheumatologists, general internists, dermatologists, hematologists, nephrologists, gastroenterologists, infectious disease experts, pulmonologists, cardiologists, geriatricians, neurologists, and ophthalmologists. Vascular surgeons and general surgeons deal with gangrene of extremities, intestines, and other organs. Some cases can be managed by family physicians. Acute treatment can be started by specialists in emergency medicine.

Diagnosing Vasculitis
Dr. Jacobs will probably suspect the diagnosis after hearing your medical history and complaints, and after a complete examination. He will probably request that additional tests be taken. Blood abnormalities which often occur when vasculitis is present include an elevated sedimentation rate; anemia, high white blood cell count, and high platelet count. There are blood tests that will identify immune complexes and whether complement levels are abnormal. These test take several days to complete. Dr. Jacobs may also order a urine analysis. If there are any symptoms that suggest heart involvement, tests that may be ordered include: EKG, ECHO cardiogram and heart scans. For lung symptoms, the physician may order a chest x-ray, obtain blood from an artery to measure the oxygen content, and schedule a pulmonary function test, in which a machine measures how well the lungs can handle air and oxygen as you breathe into it. If there are abdominal symptoms, the physician may order ultrasound or CAT scans of the organs in the abdomen, or other special x-rays to see the intestines. For brain symptoms, CAT scans and magnetic resonance images are frequently useful. Sometimes inflammation in medium and large-size arteries or veins can be seen by injecting dye into them and viewing the outlines of the blood vessels on x-ray. This procedure is called "angiography." It may be done in any area of the body. The diagnosis is most firmly made by seeing vasculitis in involved tissue. This is done by taking a biopsy of the involved tissue and examining that tissue under a microscope. Dr. Jacobs may suggest this procedure. Finally, it may be important for Dr. Jacobs to consult with other medical specialists about your case. For example, if Dr. Jacobs suspects eye involvement, and you have visual complaints which could be indicative of vasculitis, you might be immediately referred to a consulting ophthalmologist. It is very important that one physician be in charge of your case, coordinating your care and helping you with decisions. This one physician is usually your internist or family physician.

Treating Vasculitis
The choice of treatment for vasculitis depends on how severe the vasculitis is, the status of your general health, and your past reactions (positive and negative) to medications. Many cases of vasculitis do not require treatment. A few spots on the skin now and then which are from vasculitis and are not associated with any symptoms may not require any change in medications. Most physicians recommend cortisone-type medications, such as prednisone, prednisolone, or methylprednisolone (Medrol) as the usual initial treatment for vasculitis if the vasculitis is making you sick. Usually, the medication is given in one to four doses every day at first, or in higher doses such as 40-100 mg. of prednisone a day. As the vasculitis improves, your doctor may recommend a schedule to gradually lower the prednisone dose. You and your doctor always hope that the medication can be discontinued when the illness subsides. Sometimes this is possible and sometimes it is not. The vasculitis may return when the prednisone dose drops below a certain "critical" level and you will need to take some prednisone every day or every other day for many months or even years. Usually, the length of time you will need to take prednisone and the dose required cannot be predicted by the physician in advance. You and your doctor have to determine the treatment plan as the dose is changed. Unfortunately, there are several undesirable side effects of prednisone treatment, including weight gain, increased susceptibility to infections, fragile skin, weakening of bones, tendencies toward high blood pressure, and diabetes, and cataracts. Usually, you will need to take enough prednisone to keep the vasculitis from becoming disabling. In most people, however, there will be some undesirable side effects at that dosage. You may need to accept the side effects of treatment and that disease control is not perfect. An open discussion with Dr. Jacobs about the side effects is important. Some people with severe vasculitis or vasculitis that does not respond well to cortisone-type drugs will need to be treated with cytotoxic drugs. These medications kill the cells that cause inflammation in the blood vessels. The three most frequently used are azathioprine (Imuran), Methotrexate, and cyclophosphamide (Cytoxan). They are sometimes used in combination with prednisone and are often effective in suppressing vasculitis. There are also several undesirable side effects to these medications, including suppression of bone marrow, infertility, nausea, liver disease, and an increased risk of some forms of cancer. Again, the importance of controlling vasculitis must be balanced with acceptance of some of the side effects. There are some experimental procedures that have been helpful in treating some cases of vasculitis. These include plasmapheresis and a medication used to prevent organ rejection in transplant patients (cyclosporin). Experimental therapies change frequently. Dr. Jacobs can provide you with current information. Also, vasculitis is a serious illness and regular advice and supervision from your doctor is essential.

There are various outcomes for people suffering from vasculitis. For many patients, vasculitis, especially if confined to the skin, may be annoying but never life-threatening. For those individuals, life can be normal or close to it. On the other hand, a small number of unlucky people have severe vasculitis, involving major organ systems. In these cases, damage can occur so rapidly that treatment does not have time to work or the condition may be resistant to treatment. An attack of vasculitis can be fatal or permanently disabling (by a stroke, for example) for individuals so affected. For the vast majority of people with vasculitis, treatment is very effective. The vasculitis may disappear completely and never reoccur. Or, it may disappear only to reoccur later and require treatment again. It may also be suppressed but may never really go away, so that some ongoing treatment is always required. To increase prospects for a good outcome you will want to: (1) Establish a good, trusting relationship with your medical care providers; (2) Be well informed about the illness and the treatments you are receiving; (3) Carefully follow the plan made by you and your doctor; (4) Contact your physician if you think something has changed; (5) Be sure you understand what is being done, and why; and (6) Involve family members or close friends in your treatment process. Their help and support can be extremely important.