Lupus and your body

What do I need to know about vasculitis and lupus?

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)
  • large blood vessels (arteries or veins).

How Does Vasculitis Damage the Body?

Several things can happen to an inflamed blood vessel.

  • 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.
  • 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.
  • The inside of the vessel tube may become narrowed so that blood flow is reduced.
  • The inside of the vessel tube may become totally closed, usually by a blood clot which forms at the site of 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. If untreated, this can progress to gangrene.

What Causes Vasculitis?

Vasculitis can be caused by:

  • 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.
  • Antibodies that react against cells in vessel walls. In some cases of vasculitis, an antibody against the cytoplasm of neutrophils (anti-neutrophil cytoplasm antibodies, or ANCA) is the probable cause of the inflammation and damage.
  • An immune or "allergic" reaction in the vessel walls. This cause of vasculitis is more common. Substances that cause allergic reactions are called antigens (abbreviated from anti-genetic, meaning “against genetic material”). Antigens cause the body to make proteins called antibodies which attach (bind) to the antigen for the purpose of getting rid of it. Antigens and antibodies that bind together are called immune complexes. Two primary ways in which immune complexes destroy antigens are:

o     by attracting white blood cells to digest the antigen

o     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. ANCA probably react against white blood cells in vessel walls and increase the injury to vessels.

Which Diseases Are 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:

  • lupus
  • rheumatoid arthritis
  • polymyalgia rheumatica
  • scleroderma
  • granulomatosis with polyangiitis (Wegener's granulomatosis)
  • micropolyangiitis (occurs primarily in kidneys)
  • temporal arteritis
  • cryoglobulinemia
  • erythema nodosum
  • tumors
  • leukemia
  • lymphoma

How is Vasculitis Caused by Lupus?

The antigens causing the immune complexes in lupus 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.

What Are Symptoms of Vasculitis?

Vasculitis can cause many different symptoms, depending upon what tissues are involved and the severity of the tissue damage.

Some individuals will not become ill but will notice spots on their skin. Others can be very ill with systemic symptoms and major organ damage.

The symptoms in this list are grouped based on the areas of tissue in which vasculitis occurs.

Systemic symptoms can occur in many illnesses and are 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 and 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, swelling, and heat in joints, and arthritis with pain. Deformities resulting from this arthritis are rare.

Brain

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

  • headaches
  • behavioral disturbances
  • confusion
  • seizures
  • strokes

Peripheral Nerves

Peripheral nerve symptoms of vasculitis may include:

  • numbness and tingling (usually in an arm or a leg, or in fingers or toes),
  • loss of sensation and 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 a hole (a perforation) develops in the intestinal wall, peritonitis with diffuse abdominal pain may occur; 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 and symptoms of fever and cough.
  • Occasionally, inflammation can lead to scarring of lung tissue and 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 people with renal vasculitis have high blood pressure.
  • The physician will find protein in the urine and red blood cells on microscopic examination of urine (these results will be reported to the physician by a laboratory performing urinalysis).
  • The physician will also order a blood test for ANCA to see if that antibody is involved in inflaming the kidney.

Eyes

  • Sometimes, vasculitis of the eyes causes no symptoms. Usually, however, there is visual blurring which comes on suddenly and stays, and may result in some loss of vision
  • Vasculitis involving the small blood vessels of the retina (at the back of the eye) can occur in lupus, affecting the retinas’ ability to form a visual image.
  • In non-lupus types of vasculitis, such as temporal arteritis, there can be sudden loss of part or all of the vision in one eye, which is usually accompanied by severe headache.

Which Type of Physicians Know 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, needing no treatment OR
  • very severe and life-threatening OR
  • any degree in between.

Therefore, an expert should help you decide:

  • if you have vasculitis
  • if so, how serious it is
  • if and how it should be treated.

Doctors trained in many 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 (seniors)
  • neurologists (the nervous system)
  • ophthalmologists (the eyes).

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 recommended that one physician is in charge of organizing your care for two reasons: to coordinate your care, and to help you with decisions.

How is Vasculitis Diagnosed?

The diagnosis of vasculitis is based on:

  • a person's medical history
  • current symptoms
  • a complete physical examination
  • the results of specialized laboratory tests, including ANCA.

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.
  • a positive ANCA test

Blood tests can also be used to identify immune complexes or antibodies that cause vasculitis in the circulation and to measure whether complement levels are abnormal.

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:

  • chest X-ray
  • blood from an artery to measure the oxygen content
  • pulmonary function test, which uses a specialized machine to measure how well the lungs handle air and oxygen.
  • CT scan of the lungs.

Abdominal testing

If there are abdominal symptoms, the physician may order ultrasound or CAT scans of the organs in the abdomen, and/or special X-rays of the intestines.

Brain testing

If the brain is affected, the physician may order CAT scans and/or magnetic resonance images (MRI), and possibly an angiogram. MRI angiograms can be done as a combined test.

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. MRI techniques can be modified to visualize arterial and small vessel blood flow. Ultrasound can be used to see clotting in some of the larger arteries and veins that may be inflamed.

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.

Other laboratory tests

The physician may also order a urine analysis, or urinalysis.

How is Vasculitis Treated?

The choice of treatment for vasculitis depends on several factors:

  • the severity of the vasculitis
  • your general health
  • 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 corticosteroid medications as the initial treatment for vasculitis such as prednisone, prednisolone, or methylprednisolone (Medrol®).

Some people with severe vasculitis or vasculitis that does not respond well to steroid-type drugs will need to be treated with cytotoxic drugs or rituximab. Cytotoxic drugs kill the cells that cause inflammation in the blood vessels. The most frequently used are azathioprine (Imuran®), cyclophosphamide (Cytoxan®) and mycophenolate mofetil (Cellcept). Cytotoxic medications are usually used in combination with prednisone and are often effective in treating vasculitis. A biologic drug called rituximab (Rituxan®) that depletes B cells (which make autoantibodies such as ANCA) may be a good choice for treatment, often instead of cytotoxic drugs.

Experimental procedures that have been helpful in treating some cases of vasculitis include:

  • plasmapheresis
  • intravenous gamma globulin
  • cyclosporine (cyclosporin A, Neoral®, Sandimmune®) or tacrolimus (Protopic®), medications used to prevent organ rejection in transplant patients.

Experimental therapies change frequently. Your physician can provide you with current information.

What is the Prognosis for People with Vasculitis?

There are various outcomes for people with vasculitis.

For many people, 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.

A small number of people have severe vasculitis involving major organ systems. In these cases, damage may 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 permanently disabling or even fatal.

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

  • the symptoms of 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.

Medically reviewed on July 15, 2013