No data suggest that the family members of patients with vasculitis are at a substantial increased risk for developing vasculitis. Testing to try to identify vasculitis in anyone, whether a family member of a patient or not, should be driven by specific symptoms or medical problems.

The first step to find a specialist with expertise in vasculitis treatment is to ask your primary physician. Many patients with vasculitis are cared for by rheumatologists. The American College of Rheumatology has a tool on its web site to help locate a rheumatologist by geographic site and many local chapters of the Arthritis Foundation maintain lists of local rheumatologists. For physicians with expertise in vasculitis care, the Vasculitis Foundation can also offer suggestions. Some patients with vasculitis affecting specific organs need care from other specialists, such as pulmonologists (lung disease specialists) or nephrologists (kidney specialists).

This is a complicated question and raises issues about trust and confidence in one’s physician. Because of the complexities in diagnosing and treating vasculitis, patients with vasculitis should receive care directed by a physician with expertise in vasculitis. Seeking a second opinion might be considered if there is a problem making a diagnosis, or to help answer questions about therapy, especially if current therapy is not effective or complicated by adverse reactions.

"Remission" is a term used to indicate that there is no longer any detectable inflammatory disease activity. The use of the term "remission" implies that the disease may not be cured, and that relapses are possible in the future. Once a state of remission has been achieved the intensity of immunosuppressive therapy is usually reduced. This means that the dose of prednisone is reduced and that the first-line remission-induction agent (such as cyclophosphamide) is replaced by better-tolerated, milder forms of immunosuppression that are used more long-term to keep the patient in remission (such as methotrexate, azathioprine, or mycophenolate mofetil).

"Remission" does not necessarily mean that the patient is feeling perfectly well. This is because symptoms may be caused by either active inflammation associated with vasculitis, or by organ damage resulting from active disease or by side effects of medications used to put the patient in remission. "Remission" also does not mean that all serological markers of a specific form of vasculitis (such as ANCA or ESR) have completely disappeared or normalized.

The concept of "remission" is often expanded through use of the terms "partial remission", "complete remission", or "sustained remission". Partial remission means that the disease has improved but that there is still some detectable inflammatory activity in at least some of the organs afflicted by the disease before treatment was started. Complete remission means that there is no more inflammatory activity detectable in any of the affected organs. Sustained remission implies that the state of complete remission has been maintained for at least six months.

A patient can be in remission on medication or off all immunosuppressive medications. The ideal state, of course, is to have experienced a sustained complete remission lasting long enough to allow for discontinuation of all immunosuppressive therapy and maintaining that state long-term without suffering a relapse.

A disease flare represents recurrence of inflammatory activity of the underlying vasculitis after a state of remission has been achieved with treatment. Usually disease flares are milder than the original disease presentation because the patient may still be on some immunosuppressive therapy that muffles the intensity of the inflammation or because symptoms get recognized faster by the patients and their physicians, and a renewed course of remission induction therapy is implemented earlier.

Disease flares do not always occur in the same way as the disease originally presents. The rule of thumb is that the sooner a relapse occurs (the shorter the time of remission has been), the more likely the relapse will be similar to the original disease presentation. In contrast, relapses occurring after several years in stable, possibly drug-free remission have a higher likelihood to present differently and involve other organs than those originally afflicted. These patterns certainly do not always occur.

Remission means the absence of disease activity. A relapse, also sometimes referred to as a "flare," is a worsening of disease activity. The term "relapse" is used to indicate disease related inflammation that has the potential to cause organ injury and requires treatment.

The triggers for disease relapses are unknown. While many questions have appropriately been raised about factors such as infection and stress, no definitive association between these or other possible triggers have been found to be linked with disease relapses.

Not necessarily. The pattern of relapses that people will experience is very individual. Some people may experience no relapses after their vasculitis enters remission. Some people may have one relapse early on and no more, some may have multiple relapses but then have no more, and some people may have their first relapse many years after their first disease flare. Because of this it is difficult to view people with relapses as having any predictable pattern for the future. It is important that people who relapse should not feel discouraged that they are always destined to relapse in the future as this may not be the case. Ongoing monitoring by careful evaluation of new symptoms, regular physician visits, laboratory tests, and possibly imaging studies, all play important roles in the follow-up of patients with vasculitis, regardless of the number and type of relapses an individual has had.

The term "relapse" is used to indicate disease related inflammation that has the potential to cause organ injury and requires treatment. Determination of a relapse is based upon the finding of disease-related inflammation. The evidence for this comes from review of symptoms, physical examination, laboratory tests, and sometimes imaging studies. Relapses must be differentiated from damage, infection, medication side effects, or other problems that can cause similar symptoms or laboratory abnormalities. Relapse manifestations and frequency vary for each form of vasculitis and have been determined based on studies published in the medical literature.

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
It can be hard to determine relapses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). A return of sinus symptoms and asthma is very common, but these but may not represent active vasculitis. These symptoms may require treatment with prednisone and are commonly the rate-limiting features in being able to reduce the prednisone dose in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss). Relapses of vasculitis in Eosinophilic Granulomatosis with Polyangiitis are less common and have been reported in ranges from 10-30%

Giant cell arteritis
Reported relapse rates in giant cell arteritis have ranged from 50-90%.

Microscopic polyangiitis
Reported relapse rates in microscopic polyangiitis have ranged from 40-60%.

Polyarteritis nodosa
Reported relapse rates in polyarteritis nodosa have ranged from 10-30%.

Takayasu’s arteritis
Reported relapse rates in Takayasu’s arteritis have ranged from 50-80%.

Granulomatosis with polyangiitis (Wegener’s)
Reported relapse rates in granulomatosis with polyangiitis (Wegener’s) have ranged from 50-70%.

Disease activity represents the aspects of your disease that are caused by active inflammation in organs targeted by the vasculitis and respond to treatment with steroids and immunosuppressive drugs. Disease damage represents the parts of your disease that do not respond to anti-inflammatory and immunosuppressive therapy. In any particular patient, damage might include problems caused directly by your disease (such as chronic kidney problems or neuropathy causing numbness in the feet), or problems caused by the drugs used to treat vasculitis (such as diabetes or high blood pressure, both of which can be caused by steroid use).

Measuring the creatinine level in the blood provides an indicator of how well your kidneys are working. Higher creatinine levels mean worse kidney function. When you have a form of vasculitis affecting the kidneys small filtering parts of your kidneys (glomeruli) are gradually being injured by your disease. The purpose of treatment, in general, is to stop this injury, but treatment doesn’t restore the kidneys to their previous, healthy state. Thus, even after the vasculitis in the kidney has been quieted, the creatinine may remain elevated. For many patients, a stable creatinine level is an excellent indicator that the treatment is working, and that your kidneys are not being subjected to further injury by your disease.

There is no simple answer. Specific data are not available and it is hard to answer as there are many different types of vasculitis, some of which are mild and others which are more severe. Some forms of vasculitis can affect vital organs and be life-threatening when the disease is active. Vasculitis can also cause damage to organs that can affect overall life expectancy. In addition, use of medications to treat vasculitis that suppress the body’s immune system can increase the risk of infection. It is best to address this with your own physician to take into account your particular type of vasculitis and problems that might have arisen from the disease and its treatment.

This question is difficult to address in general terms because there are a lot of individual factors that influence the need for specific laboratory tests. In principle, serial laboratory testing over time has two major goals. First, laboratory testing should screen for disease activity. Second, laboratory testing should look for toxicity of the medications used to control the disease activity.

The following battery of tests is usually sufficient for most types of vasculitis; markers of inflammations (erythrocyte sedimentation rate and C-reactive protein), a complete blood count (with differential cell count), a blood chemistry panel (that includes liver function tests and kidney function tests), and a urinalysis with urine microscopy (for forms of vasculitis that frequently involve the kidneys). The frequency of these tests depends on the individual situation (type of disease, organ system involvement, medications used, whether remission has been achieved, and the duration of remission, etc.)

In addition to laboratory testing, your doctor may also choose certain imaging studies such as chest x-ray, chest CT (CAT) scan, MR angiograms (MRI), or others, to follow specific disease manifestations in specific forms of vasculitis.

Increases in the sedimentation rate and/or C-reactive protein do not predict disease relapses. The sedimentation rate, more formally termed the erythrocyte sedimentation rate, also known as the "ESR" or "sed rate," and the C-reactive protein (or "CRP") are non-specific markers of inflammation. They are usually, but not always, elevated at the time of vasculitis relapse, but not necessarily before the relapse. Since they are non-specific markers, they can be elevated in any condition associated with inflammation ranging from any type of infection to surgical procedures. The sedimentation rate can also high in patients with renal insufficiency or anemia. Decisions about treatment should not be made based on the sedimentation rate and C-reactive protein alone.

This is a general question that cannot be answered specifically without knowing the patient’s individual situation. A well-balanced diet following recommendations of the American Heart Association is usually sufficient. In patients with high blood pressure, a low-salt diet maybe necessary. Patients with renal insufficiency will have specific restrictions of protein and potassium intake. Specific dietary recommendations also apply to patients with diabetes, which in turn may be worsened by the use of glucocorticoids. A consultation with a dietitian who can factor in an individual patient’s specific needs is certainly a good option for some patients.

Long-term treatment with prednisone, even at relatively low doses, is associated with bone loss and is considered a risk factor for bone fractures even with a normal bone density test. All patients treated with prednisone should be on supplemental calcium (1000-1200 mg daily) and vitamin D (800 IU daily) therapy unless contraindicated. The decision regarding therapy with other medications can be made after a more detailed individual risk profiling for fracture.

There is no clear data on alternative therapies for vasculitis. Since we do not know the immunologic effects of medications such as over-the-counter supplements, they may be associated with unwanted effects related to stimulation of the immune system.

Progressive multifocal leukoencephalopathy, also known as "PML," is a brain infection that is caused by the JC virus. Exposure to this virus is common, but it generally causes problems only in patients who have been treated with aggressive immunosuppressive therapy, such as patients receiving chemotherapy for cancer. PML has also been reported in patients receiving drugs to treat rheumatic or autoimmune diseases, such as lupus and rheumatoid arthritis, although this is extraordinarily rare. PML is diagnosed by brain biopsy or by looking for evidence of the JC virus in cerebrospinal fluid (CSF) obtained by lumbar puncture (spinal tap), using a technique called polymerase chain reaction (PCR). Because neither brain biopsy nor CSF examination are perfect, we generally recommend that such tests are performed only when the suspicion of PML is very high.

Intravenous immunoglobulin (IVIG) is not often used to treat vasculitis. IVIG is the purified antibody-containing part of the blood proteins obtained from blood donations. IVIG is most often used in treating vasculitis when a patient with active vasculitis also has an infection. Unlike most other treatments used in treating vasculitis, IVIG does not suppress the immune system, and hence does not make it more difficult to get over infections. IVIG may also be used when other standard treatments for vasculitis have failed or have not been tolerated well by patients. Treating physicians usually decide on a case-by-case basis how many days of IVIG to give. IVIG is usually given in monthly courses repeated courses monthly for 3-6 months.

Yes, patients with vasculitis should get a flu shot every fall. The treatments taken by most patients with vasculitis make them at higher risk of getting infections like influenza A, which could be severe. Patients should get the regular flu shot, which is a "killed virus" vaccine an unable to cause infection, rather than the nasal spray version, which is a "live attenuated" virus and able to cause infection in people who receive it, a special problem for patients taking immune suppressing treatments.

Yes, other health issues have to be kept in mind in patients who have vasculitis. Some can be related to vasculitis directly and others can be related to side effects of treatment of vasculitis. Those related to vasculitis directly include things such as stroke, heart attacks, nerve damage causing weakness, lung damage causing shortness of breath and cough, and kidney damage that may lead to swelling of the legs and dialysis. Issues related to treatment include such things as osteoporosis, high blood pressure, high cholesterol, infections, and development of certain type of cancers.

Severity can have many definitions. From an individual patient’s perspective, severity can be viewed as the amount of impact the vasculitis has on their overall functioning or the functioning of an individual organ system. From the perspective of your treating physician or a researcher involved in vasculitis, severe disease usually is taken to mean involvement with potentially life-threatening or organ-threatening disease. Involvement of the kidneys, brain, eyes, heart, and intestines, as well as bleeding into the lung, is typically considered features of "severe" disease.

The earlier that vasculitis is identified and treated the greater the chance of recovery from nerve damage. Unfortunately, many times recovery may only mean a partial recovery that may take many months or years to happen.

A clinical trial is a research study to answer specific questions about new therapies or new ways of using known treatments. Clinical trials are used to determine whether new drugs or treatments are both safe and effective.

If there is a contact person listed with the trial information you can talk to them yourself, or have your doctor call on your behalf.

The VCRC has a number of research studies and clinical trials for specific diseases and you can find out more by visiting the VCRC Find a Trial page. There is also information available on all clinical research studies being conducted in the United States on the ClinicalTrials.gov website.

The VCRC Contact Registry is a method by which patients with Vasculitis can register themselves with the VCRC in order to be contacted in the future about clinical research opportunities and updates on the progress of the VCRC research projects. The contact registry is anonymous and free of charge. You can join the registry by visiting the VCRC Contact Registry page. Patients with Vasculitis are strongly encouraged to join the VCRC Contact Registry.

We realize that not everyone who qualifies for the research projects of the VCRC will be able to travel regularly to a VCRC Center to participate in studies. However, one option is be sure to enroll in the VCRC Patient Contact Registry. Not only will this ensure you are kept updated on new projects, but also new projects may be launched that you can participate in even from afar.

It would be great to be able to study every type of vasculitis since there are unmet scientific and clinical needs for each disease. However, there are limits to our research resources. We decided to concentrate on 6 different types of vasculitis, representing small vessel (Granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis, and Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)), medium vessel (polyarteritis nodosa), and large vessel (giant cell arteritis and Takayasu’s arteritis). These diseases represent a broad spectrum of vasculitis types and were disease in which we thought it was feasible to conduct research. We may expand the number and type of diseases under study within the Consortium, depending on funding and research ideas.

It is important to remember that it is quite likely that new findings in one type of vasculitis can be beneficial in our understanding of the cause or how to treat other types of vasculitis.

You find a list of support group’s leaders in the United States and internationally by visiting the Vasculitis Foundation’s website: vasculitisfoundation.org

The Vasculitis Foundation provide detailed information and links to resources on each type of vasculitis: www.vasculitisfoundation.org

If you have additional vasculitis-related questions that were not addressed in the list above, please submit them using the email link provided below. We will answer your question within 3-4 weeks.

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