Frequently Asked Questions

What causes vasculitis?

The causes of most forms of vasculitis remain unknown. Infections are strongly suspected of playing a role in many forms, however. For example, hepatitis B (a virus) causes some cases of polyarteritis nodosa, and hepatitis C (another virus) is the cause of most cases of cryoglobulinemia. Bacterial infections have been suspected of playing a role in Wegener’s granulomatosis, and some patients with Wegener’s that is limited to the upper respiratory tract are treated only with an antibiotic, Bactrim (trimethoprim/sulfamethoxazole). A general theory that applies to many types of vasculitis is that the disease results from the occurrence of a particular infection in a person whose genes (and other factors) make him/her susceptible to developing vasculitis.

What is going to happen to me?

The course of vasculitis is often difficult to predict. Some types of vasculitis tend to occur only once and not return. Other types are prone to recurrences. For all patients with vasculitis, it is essential to be evaluated by physicians experienced in the treatment of these diseases. Most forms of vasculitis are treatable (see below), and many patients may achieve remissions through treatment. The trick is often in balancing the types of medications necessary to control the disease and the risk of side–effects that those medicines often bring. A primary aim of several ongoing new studies in vasculitis is to find drugs that help maintain remissions.

Is vasculitis curable?

Most forms of vasculitis are treatable if detected early enough, before substantial organ damage has occurred. While often effective, however, the treatments remain imperfect, and require improvement. Further research is needed in all forms of vasculitis. Greater knowledge of these diseases will lead to better treatments and, some day, to cures.

Will my children or other family members get it?

Vasculitis is not contagious. Despite the fact that infections probably play a role in many forms of vasculitis, one cannot acquire vasculitis from contact with a vasculitis patient. In addition, despite the fact that genes probably play a role in susceptibility to some forms of vasculitis, it is unusual for vasculitis to occur in more than one member of the same family. Thus, vasculitis is not a heritable disorder. All of these points illustrate the fact that the causes of vasculitis are complex. In all likelihood, patients develop vasculitis because of the simultaneous occurrence of multiple risk factors, most of which remain poorly understood.

Does diet affect vasculitis?

This is one of the most commonly-asked questions by patients with vasculitis. All patients want to do whatever is within their power to help treat their disease. Unfortunately, there is presently no evidence that a person’s diet affects susceptibility to vasculitis, or that consuming or avoiding certain foods or beverages affects the course of the disease. In general, we advocate eating a balanced, “heart–healthy” diet. Avoidance of excessive calories may be very important, particularly in patients on steroids who are at risk for weight gain. We believe that some vitamins are appropriate, but are skeptical about many “alternative” health remedies. Many of these are poorly-tested, unregulated, and expensive. Some may even be dangerous.

Will my vasculitis return?

After patients achieve remission from their vasculitis, it is logical for them to wonder if their disease will ever return. The answer, which is often difficult to give with certainty, depends in large part on the patient’s specific type of vasculitis. For example, some types of vasculitis, such as Henoch-Schönlein purpura (HSP) or vasculitis caused by a medication, tend to be self-limited and resolve on their own. Other forms of vasculitis (e.g., Buerger’s disease, a disease strongly associated with cigarette smoking) resolve with institution of the definitive treatment: smoking cessation. Still other forms behave less predictably, never coming back in some patients but demonstrating a tendency to recur eventually in up to half or more of all cases. Wegener’s granulomatosis, giant cell arteritis, Takayasu’s arteritis, microscopic polyangiitis, and other types of vasculitis fall into the category of diseases that often “flare” following the achievement of remission. Sometimes flares occur when patients go off of their treatments. Other times vasculitis flares when treatments are lowered in attempts to avoid side-effects.

At the present time, the ability of doctors to predict who will suffer disease flares and who will maintain in long-term remissions (or be cured) needs refinement. Progress in this area will come through research.

How should I guard against the occurrence of a disease flare?

We believe that several points are worth keeping in mind:

First, the symptoms of flares are usually very similar to the ones patients had when their diseases began. If headaches signaled the beginning of giant cell arteritis before, then the recurrence of headaches may indicate a disease flare. If leg ulcers began as painful red lumps on the leg the first time, then the return of painful red lumps may mean that vasculitis is back. Patients must become experts about their own manifestations of vasculitis so that they can recognize them immediately, consult their doctors, and begin appropriate treatment before serious damage occurs.

Second, patients must learn to know their own bodies, and report to their doctors any feelings or findings that are unusual. New symptoms may not only indicate a vasculitis flare, they may also herald complications of treatment such as infections.

Finally, because vasculitis treatments require careful monitoring by doctors, patients should discuss any changes in treatment with their physicians. Increasing or decreasing medications without consulting a physician may lead to trouble.

Why do I have to have bloodwork checked frequently?

It’s simple (you’ve probably heard us say it before): “A watched kettle doesn’t boil”. The more detailed answer is that in the management of patients with vasculitis, we are concerned not only with treating the disease, but also with preventing side effects of treatment. The lab work that we assess regularly is designed to help us accomplish both of these goals.

What types of tests do we check?

Regardless of the type of vasculitis and the exact type of medication that a patient takes, similar types of tests must be monitored. These tests are:

  • 1) a complete blood count;
  • 2) tests of kidney function including a urinalysis; and
  • 3) liver function tests.

The table below outlines the importance behind checking each of these tests. Sometimes other tests are required, too, for special circumstances, as when a patient is on a “blood thinner” such as coumadin.

How often should my blood be checked?

This depends on the specific medicine or medicines that you take. Patients on cyclophosphamide (Cytoxan) should have their counts checked every 2 weeks. Patients on most other kinds of medications used to treat vasculitis (Methotrexate, Azathioprine) usually only need to have their blood work checked monthly. If some laboratory tests are abnormal or nearly so, then more frequent monitoring may be required.

Type of Test What should be checked Why?
Complete Blood Count (“CBC”)
  • White blood cells (WBC)
  • Platelets
  • Hematocrit
  • Low WBC count may lead to infections.
  • Low platelets may cause bleeding.
  • Low hematocrit means insufficient oxygen-carrying capacity of the blood.
Kidney Function
  • Creatinine
  • Blood Urea Nitrogen (BUN)
  • High creatinine and BUN indicate that the kidneys are not performing their blood-cleansing function properly.
Urinalysis
  • Protein Level
  • Red Blood Cells
  • Normal urinalyses have no protein and no blood.
  • The presence of protein and/or blood in the urine may indicate active vasculitis in the kidneys (or damage to the bladder from cyclophosphamide).
Liver Function
  • Albumin
  • Aspartate aminotransferase(AST)
  • Alanine aminotransferase (ALT)
  • Often a good indication of overall health.
  • Elevated AST/ALT levels indicate inflammation in the liver (usually caused by medications).