Lupus Vasculitis and Blocked Blood Vessels

Adapted from a talk at The SLE Workshop at Hospital for Special Surgery


Michael D. Lockshin, MD

Attending Rheumatologist, Hospital for Special Surgery
Professor of Medicine, Weill Cornell Medical College
Director, Barbara Volcker Center for Women and Rheumatic Disease

  1. Warning About Misuse of the Term
  2. What is Vasculitis?
  3. What Vasculitis is Not
  4. When Does Vasculitis Occur in Rheumatic Disease?
  5. When is Vasculitis an Important Complication of an Autoimmune Disease?
  6. Diseases in Which Vasculitis is the Specific Illness
  7. Vasculitis by the Size of the Vessel
  8. Small Vessel Vasculitis
  9. Medium Vessel Vasculitis
  10. Large Vessel Vasculitis
  11. Summary

 

Vasculitis means inflammation of the blood vessels. (Vasc refers to blood vessels and itis means inflamation). Vasculitis is a problem that can arise independently of other illnesses, or it may co-exist with lupus or other autoimmune diseases. When it exists in lupus, it may simply confirm the diagnosis, causing no problems, or it may represent a change in the course of the lupus, with vasculitis as a serious complication.

Thus, vasculitis may mean many things. If a doctor says you have vasculitis, ask what that really means - what disease process is going on and what it means for you.

Warning About Misuse of the Term "Vasculitis"

Vasculitis should not be confused with vasculopathy, which simply means something is wrong with the blood vessels, although it's usually not vasculitis. Some people use these words interchangeably, which is wrong. (Some people, even physicians and especially on the Internet, use the term "vasculitis" very loosely.)

You may see statistics about vasculitis on the Internet that are very frightening, but they don't provide any information on your situation. The term vasculitis is used ten times as often as it should be by people who are not really referring to this disease. Unfortunately, even some doctors often use the term vasculitis to mean "person with autoimmune disease and blood vessel abnormality that I don't completely understand."

If your doctor says you have vasculitis, ask specifically what he/she means before you go to the Internet!

What is Vasculitis?

Vasculitis is blood vessel inflammation that causes fever, pain, local tenderness, and other evidence of blocked blood vessels.

When a blood vessel becomes inflamed and narrowed, blood supply to that area can become partially or completely blocked. Complete blockage is called occlusion; it causes the vessel wall to swell and makes things stick to the wall -- so a clot forms. When vasculitis interferes with circulation in any part of the body, it causes local tenderness and pain. If the blood vessels are close to the skin, characteristic rashes occur. Depending on where the blockage occurs, almost any organ in the body can be affected. (Note: Vasculopathy can also block blood vessels, but it does not cause the fever, pain, and local tenderness associated with vasculitis.)

While vasculitis may involve arteries (the thick muscular vessel that carries blood away from the heart) and veins (the thinner, less muscled vessels that carry blood toward the heart), it is rare for both arteries and veins to be involved at the same time.

What Vasculitis is Not

Many problems that block blood vessels looks like vasculitis, and doctors often jump the gun and call them vasculitis, but greater care is needed to find out what's really going on. Among the diseases involving blocked blood vessels that are not vasculitis:

  • Atherosclerosis (hardening of the arteries)
  • Growths on the heart valves that break off, especially those due to infection
  • Excessive blood clotting (antiphospholipid syndrome)
  • Vessel spasm, especially due to drugs (legal and illegal)

When Does Vasculitis Occur in Rheumatic Disease?

All of the rheumatic diseases involve some level of underlying vasculitis. That includes lupus, rheumatoid arthritis (RA), scleroderma, and dermatomyositis.

If you biopsy a swollen joint in RA, you routinely find vasculitis. That finding is used to confirm the diagnosis, but it doesn't mean anything important is happening. It just suggests that one of these autoimmune diseases is present. Vasculitis is a common finding in these diseases, important in diagnosis, but it doesn't necessarily mean anything more. It may never be a problem!

When is Vasculitis an Important Complication of an Autoimmune Disease?

There are times in lupus and RA when the disease takes a different course in the presence of vasculitis. You start getting sicker and developing a fever, both of which are clues to the physician that there has been a change in the course of illness. Now we say, "This is lupus complicated by vasculitis" or "This is rheumatoid arthritis complicated by vasculitis." The disease has changed its character and usually needs more vigorous treatment.

Diseases in Which Vasculitis is the Specific Illness

  • Polyarteritis nodosa, which affects middle aged people
  • Temporal arteritis or giant cell arteritis, which affects arteries in the head of older people
  • Allergic vasculitis, which is an abrupt reaction that sometimes follows infection
  • Takayasu's arteritis, which is a relatively rare disease of young women, largely in Asia, involving large blood vessels
  • Wegener's granulomatosis, which affects the kidneys, lungs, and blood vessels

These specific kinds of vasculitis are treated differently, depending on the underlying problem.

Vasculitis by the Size of the Vessel

Vasculitis is best discussed in terms of which blood vessels are involved: the small, medium, or large vessels. The large vessels are those that lead from your heart (namely, the aorta and the arteries that go across the shoulders and down to the hips).

Small Vessel Vasculitis

This is the most common form of vasculitis seen in lupus and is fairly common in dermatomyositis and scleroderma, but less common in rheumatoid arthritis. The small vessels are at your fingertips and inside your organs, such as the kidneys.

Some signs of it can be seen with a small lens that shows tiny broken vessels in the cuticle and hemorrhages along the edge or under the nail. Or on other parts of the skin, especially the legs, you see little red dots with black centers that occasionally cause a burning sensation. This small vessel vasculitis is not particularly threatening. It is a manageable but annoying category of the disease, with fairly mild symptoms that may not need treatment. Some people don't even know that they have the evidence of it. However, when the blood supply to the nerves in the feet are cut off by vasculitis, they may develop numbness.

Another type of small vessel vasculitis we see in people with lupus and rheumatoid arthritis causes kidney inflammation called glomerulonephritis. Small vessel vasculitis can also be seen in children as palpable purpura, which causes a rash. It may come and go and be treated only when it's causing problems. In addition, small vessel vasculitis can be seen in severe allergies and infections; when you treat the underlying cause, such as the infection, the vasculitis goes away.

Diagnosis of small vessel vasculitis can be made by an experienced clinician simply by observation. Here, the question is not "Is it vasculitis?" but "What's causing this vasculitis?" Less experienced physicians want to do blood tests, skin biopsy, angiography, and x-rays, all of which are not useful for this type of small vessel vasculitis. Only a kidney biopsy may be needed in some circumstances.

What does it mean when small vessel vasculitis occurs in someone with long-term lupus or RA? You do not treat the vasculitis itself; you have to look at the whole patient and ask why has this complication has occurred. It could be an allergy or an infection, which should be found and treated. Small vessel vasculitis should not be ignored because it is important, but it's not life-threatening. If allergy or infection have been ruled out, treatment with corticosteroids (prednisone) or other immunosuppressants is more or less negotiable depending on what the patient wants to tolerate in terms of side effects.

Medium Vessel Vasculitis

These vessels include those that lead down your arms and legs, and also to your heart and brain, and even those supplying blood to your intestines. Medium vessel vasculitis is what physicians mean when they call vasculitis a serious disease. (In polyarteritis nodosa, for example, it tends to be very dramatic. People say they have never felt worse in their lives.) Medium vessel disease can occur in lupus and RA, but it is very rare.

The symptoms of medium vessel vasculitis include: fever; severe muscle aches; sudden loss of power of specific muscles (perhaps a toe starts dragging); severe abdominal pain; sudden loss of circulation in a finger or toe that turns totally black; stroke; or heart attack. Again, the diagnosis can be made at bedside based on symptoms. Blood tests are useful for making the diagnosis. Sometimes a muscle biopsy or nerve conduction studies are done. These procedures provide information to confirm a diagnosis. The type also must be identified; it may be caused by infection, such as hepatitis B, or it may be polyarteritis nodosa alone, or it may be a complication of an autoimmune disease.

Medium vessel vasculitis can be life-threatening because the blocked vessels going to the heart can cause a heart attack, or those going to the brain may cause a stroke. Survival depends on immediate recognition of the disease and immediate and vigorous treatment. When medium vessel vasculitis occurs, treatment is not negotiable. High dose steroids and cytoxan are needed to reverse it as soon as possible.

Large Vessel Vasculitis

We don't see large vessel vasculitis in lupus and rheumatoid arthritis. When the large blood vessels develop vasculitis, it's always an independent disease, such as Takayasu's or giant cell or cranial arteritis. This type of vasculitis has completely different symptoms, and it requires a bit of cleverness to figure out what's going on. It's not immediately threatening, but it can do a lot of damage if not treated for long periods. Treatment is aimed at preventing disability, such as the blindness that may be caused by giant cell arteritis.

Summary

Vasculitis is inflammation of the blood vessels; vasculopathy is everything else that may go wrong with blood vessels. Vasculitis occurs as a regular part of rheumatic disease, or vasculitis may occur as its own illness. Many other problems may mimic vasculitis.

Vasculitis may be serious, but it is not serious in most cases in people with lupus and other rheumatic diseases. Trivial vasculitis in rheumatic disease, particularly of the small vessels, may not even require any change in treatment. However, when serious vasculitis occurs, very aggressive therapy is necessary, depending on the cause. In serious vasculitis related to autoimmune diseases such as lupus, aggressive therapy with prednisone and other immunosuppressants is essential.


Learn more about the SLE Workshop, a free support and education group held monthly as HSS.

Summary of a presentation given at The SLE Workshop, a free support and education group held monthly for people with lupus and their families/friends. Summary by Diana Benzaia.

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