Lupus and Vasculitis

What Is Vasculitis?

Vasculitis is an inflammation of the blood vessels. Inflammation is a condition in which tissue is damaged by blood cells entering the tissues. In inflammatory diseases, these cells are mostly white blood cells. White blood cells circulate and serve as our major defense against infection. Ordinarily, white blood cells destroy bacteria and viruses. However, they can also damage normal tissue if they invade it.

Vasculitis can affect

• very small blood vessels (capillaries),
• medium-size blood vessels (arterioles or venules), or
• large blood vessels (arteries or veins).

How Does Vasculitis Damage The Body?Several things can happen to an inflamed blood vessel.

1. If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. These areas will appear as small red or purple dots on the skin.
2. If a larger vessel is inflamed, it may swell and produce a nodule which may be felt if the blood vessel is close to the skin surface.
3. The inside of the vessel tube may become narrowed so that blood flow is reduced.
4. The inside of the vessel tube may become totally closed, usually by a blood clot which forms at the site of inflammation.
5. 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 this can progress to gangrene.

What Causes Vasculitis?

Vasculitis can be caused by

1. infection of the blood vessel walls
   • This is rare. 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 in the process.
   • This is a serious condition and requires prompt antibiotic treatment.
2. an immune or "allergic" reaction in the vessel walls.
   • This cause of vasculitis 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.

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
2. by activating other body substances to help destroy the antigens.

• Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.
• It is likely that some white blood cells (cytotoxic cells) which kill infectious agents can also accidentally damage blood vessels and cause vasculitis. 

Vasculitis Caused By Lupus 

In these cases, the antigens causing the immune complexes are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens. A recently discovered antibody, ANCA (anti-neutrophil cytoplasm antibody), can cause vasculitis in some individuals.

Diseases Associated With Vasculitis

Vasculitis can occur in many different illnesses. Vasculitis can also occur by itself without any obvious associated infection or other illness. Some of the illnesses that can cause vasculitis are:

• systemic lupus erythematosus
• rheumatoid arthritis
• polymyalgia rheumatica
• scleroderma
• Wegener's granulomatosis
• temporal arteritis
• cryoglobulinemia
• erythema nodosum
• tumors
• leukemia
• lymphoma 

Symptoms Of Vasculitis

Vasculitis can cause many different symptoms, depending upon:

1. what tissues are involved
2. the severity of the tissue damage.
3. Some patients are not ill but notice occasional spots on their skin.
4. 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 (this set of complaints can occur in many illnesses and is not specific to vasculitis)

• fever
• generally feeling bad (malaise)
• muscle and joint pain
• poor appetite
• weight loss
• fatigue.

Skin 

Common vasculitis skin lesions are:

• red or purple dots (petechiae), usually most numerous on the legs
• larger spots, about the size of the end of a finger (purpura), some of which look like large bruises
• Less common vasculitis lesions are:
• hives
• an itchy lumpy rash
• painful or tender lumps.

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)
• gangrene of fingers or toes.

Joints

• aching in joints and arthritis with pain
• swelling and heat in joints
• deformities resulting from this arthritis are rare.

Brain 

Vasculitis in the brain can cause many problems, from mild to severe, including:

• headaches
• behavioral disturbances
• confusion
• seizures
• 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
• loss of strength (especially in the feet or hands).

Intestines

• Inadequate blood flow in the intestines can cause cramping 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 (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.
• Heart attacks rarely occur as a result of vasculitis.

Lungs

• Vasculitis in this tissue can cause pneumonia-like attacks with:
  • chest x-ray changes that look like pneumonia
  • symptoms of fever and cough.
• Occasionally, inflammation can lead to scarring of lung tissue with chronic shortness of breath.

Kidneys

• Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis.
• Vasculitis in the kidneys 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 at the back of the eye.
  • The cells of the retina 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.
  • A person may even lose a portion of their vision.
• In other non-lupus types of vasculitis, such as temporal arteritis:
  • There is sudden loss of part or all of the vision in one eye
  • This is usually accompanied by severe headache.

Diagnosing Vasculitis

The diagnosis of vasculitis is based on:

1. a person's medical history
2. current symptoms
3. a complete physical examination
4. the results of specialized laboratory tests.

Blood testing

Blood abnormalities that often occur when vasculitis is present include:

• an elevated sedimentation rate
• anemia
• a high white blood cell count
• a high platelet count.

Blood tests can also be used to:

• identify immune complexes or antibodies that cause vasculitis in the circulation
• measure whether complement levels are abnormal.

These blood tests take several days to complete.

Heart testing

If there are any symptoms that suggest heart involvement, tests that may be ordered include:

- EKG (electrocardiogram)- ECHO cardiogram- heart scans.

Lung testing

For lung symptoms, the physician may order:

- a chest x-ray- blood from an artery to measure the oxygen content- a pulmonary function test, which uses a specialized machine to measure how well the lungs handle air and oxygen as you breathe into it.

Abdominal testing

If there are abdominal symptoms, the physician may order:

- ultrasound or CAT scans of the organs in the abdomen- special x-rays to see the intestines.

Brain testing

For brain symptoms, the doctor may use:

- CAT scans- magnetic resonance images (MRI).

Blood vessel testing

The physician can use a procedure called angiography to see if there is inflammation in medium and large arteries or veins. This can be done in any area of the body by injecting dye into the arteries or veins and viewing the outlines of the blood vessels on x-ray.

Tissue testing (biopsy)

The diagnosis of vasculitis is most firmly made by taking a biopsy of the involved tissue and examining that tissue under a microscope. Your physician may suggest this procedure.

Referrals to specialists

Finally, it may be important for your physician to consult with other medical specialists about your case.

For example, if your physician is a rheumatologist and you have visual complaints which could be indicative of vasculitis, you may be referred to an ophthalmologist.

However, it is very important that one physician be in charge of your case for two reasons:

1. to coordinate your care
2. to help you with decisions.

Treating Vasculitis

The choice of treatment for vasculitis depends on several factors:

1. the severity of the vasculitis
2. your general health
3. your past reactions (positive and negative) to medications.

• Many cases of vasculitis do not require treatment.
  • For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.
• Most physicians recommend cortisone-type medications as the initial treatment for vasculitis., such as:
  • prednisone
  • prednisolone
  • methylprednisolone (brand name: Medrol)
• Some people with severe vasculitis or vasculitis that does not respond well to cortisone-type drugs will need to be treated with cytotoxic drugs.
  • Cytotoxic drugs kill the cells that cause inflammation in the blood vessels. The two most frequently used are:
    1. azathioprine (brand name: Imuran)
    2. cyclophosphamide (brand name: Cytoxan).
  • Cytotoxic medications are usually used in combination with prednisone and are often effective in treating vasculitis.
• Experimental procedures that have been helpful in treating some cases of vasculitis include:
  • plasmapheresis
  • intravenous gamma globulin
  • cyclosporin, a medication used to prevent organ rejection in transplant patients.
  • Experimental therapies change frequently. Your physician can provide you with current information. 

Consulting Your Physician About Vasculitis

If you suspect that you or a friend or relative has vasculitis, a physician should be consulted 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 you decide:

1. if you have vasculitis
2. if so, how serious it is
3. if and how it should be treated.

Doctors trained in several different specialties are taught to recognize and treat vasculitis. These include:

• rheumatologists (joints and muscles)
• general internists (the adult body)
• dermatologists (skin)
• hematologists (blood)
• nephrologists (kidneys)
• gastroenterologists (the intestinal tracts, stomach, etc.)
• infectious disease experts
• pulmonologists (lungs)
• cardiologists (heart)
• geriatricians (the elderly)
• neurologists (the nervous system)
• ophthalmologists (the eyes).

Outcome

There are various outcomes for people suffering from vasculitis.

1. 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.
2. On the other hand, a small number of 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 for affected individuals.

For the vast majority of people with vasculitis, treatment is very effective. However:

• the vasculitis may disappear only to reoccur later and require treatment again OR
• the vasculitis may be suppressed but never really go away, so that some ongoing treatment is always required.

From: http://www.lupus.org/webmodules/webarticlesnet/templates/new_aboutintroduction.aspx?a=105&z=9&page=1

Related Information

On this web site:MedicationsSteroidsImmune Suppressing Drugs

On the Internet:MedlinePlus: VasculitisThe Cleveland Clinic: What You Need to Know About Central Nervous System VasculitisThe Johns Hopkins Vasculitis Center