7 Things You Should Know About Vasculitis

Vasculitis is a family of diseases many people haven’t heard of. In recent years, however, this has started to change — good news for people with vasculitis and the doctors who care for them.

Vasculitis refers to inflammation of the blood vessels. Vasculitis can occur in a number of different ways. It can occur as  a secondary condition to an underlying disease or exposure. It also can be a primary illness for which the cause is unknown and where the blood vessel inflammation injures the body’s organs.

Vasculitis as a primary disease is uncommon, ranging from forms that affect 26 people per 100,000 to those which occur in less than 1 person per million, says rheumatologist Carol Langford, MD, MHS. Dr. Langford is Director of the Center for Vasculitis Care and Research at Cleveland Clinic.

Awareness is important

Although vasculitis is rare, awareness of the disease and its symptoms is important, Dr. Langford says. That’s because treatment exists for almost all forms of vasculitis and is more effective in preventing organ damage when diagnosed early.

“In most cases, treatment can cause the disease to go into remission, which means that the condition isn’t active and is no longer causing organ or tissue injury,” Dr. Langford says.

However, for many forms of vasculitis, the illness can return or relapse. While this remains one of the main challenges in managing vasculitis, ongoing monitoring and active communication between patient and physician play a critical role in detecting and minimizing relapses should they occur.

Unfamiliar disease

Because vasculitis can often share symptoms and signs with other diseases, establishing a diagnosis of vasculitis often is difficult, Dr. Langford says.

In addition, because vasculitis is uncommon, patients can benefit from an evaluation at a facility, such as the Center for Vasculitis Care and Research at Cleveland Clinic, that specializes in evaluating and treating the disease, Dr. Langford says.

There, a team that consists of a rheumatologist and other physicians from a diverse range of medical specialties can develop a comprehensive plan based on that individual patient’s vasculitis and the organs or vessels that the disease is affecting.

“If a patient lives a distance from a vasculitis center such that travel for regular visits is difficult, physicians can work together with the patient’s home medical team in optimizing that person’s care,” Dr. Langford says.

What you need to know

Here are seven things you should know about vasculitis:

  1. Vasculitis is inflammation of blood vessels. The body’s immune system regulates inflammation.
  2. Vasculitis is a family of multiple different diseases. The types of vasculitis differ in whom they affect and the organs they involve. Some forms are mild. Others are more severe.
  3. Vasculitis can affect any of the body’s blood vessels. In vasculitis, the blood vessel walls can thicken, leading to vessel narrowing or blockage. If the flow in a blood vessel with vasculitis reduces or stops, the tissues that receive blood from that vessel begin to die. Vasculitis can also weaken blood vessels, leading to enlargement of the vessel (called an aneurysm) or disruption of the blood vessel wall, with bleeding into the surrounding tissue. In some forms of vasculitis, inflammation can occur in tissues other than blood vessels.
  4. The cause of most forms of vasculitis is unknown.
  5. Symptoms of vasculitis vary. They can include nasal congestion joint pain, mouth ulcers, hearing loss, skin lesions, headache, vision problems, numbness, weakness, cough, shortness of breath, fever, weight loss and many others symptoms.
  6. Vasculitis is treatable. The type of treatment will depend on the form of vasculitis, the affected organs and disease severity. The main goal of treatment is to reduce inflammation in the affected blood vessels and tissues. Doctors aim to reduce or halt the immune response that is causing the inflammation.
  7. Research is actively ongoing to develop more effective forms of treatment and ultimately to understand the causes of vasculitis. For example, research is under way at the Cleveland Clinic by investigators within the Center as well as with collaborators throughout the world. Additionally, the National Institutes of Health funds the Vasculitis Clinical Research Consortium, in which the Cleveland Clinic participates.

Stress triggers disease flares in patients with vasculitis

In patients with a devastating form of vasculitis who are in remission, stress can be associated with a greater likelihood of the disease flaring, according to a new study by investigators at Hospital for Special Surgery (HSS).

This is the first study to suggest that mental health is a risk factor in patients with vasculitis, a group of autoimmune disorders characterized by the inflammatory destruction of blood vessels. The study, in a form of the disease known as Wegener’s granulomatosis (WG), will be presented on Nov. 8 at the American College of Rheumatology’s annual meeting.

“When this disease flares, people can be really sick. It often affects the lungs, kidneys, sinuses and nerves. It can cause fevers and rashes. People can die from this illness. It is a very robust, active, inflammatory disease when it is active,” said Robert Spiera, M.D., director of the Vasculitis and Scleroderma Program at HSS, who led the study. “When patients are in remission, however, they can do very, very well.”

He says that doctors caring for patients with this disease should be attentive to their psychological health. “This study points out that mental health should be part of your medical assessment,” said Dr. Spiera. “You should pay attention to the patient’s mental well being and be more aggressive about intervening if a patient is in a bad place. Make sure that patients take it seriously.”

Prior to this report, a few small studies had suggested that psychological stress can trigger flares of lupus, another autoimmune disease, and doctors have observed that WG patients often say that stress in their lives, caused by perhaps a death of someone close or losing a job, made their disease flare. To investigate this anecdotal evidence in a more quantifiable way, researchers at HSS conducted a retrospective analysis of data from the Wegener’s Granulomatosis Etanercept Trial (WGET). The primary objective of this randomized, placebo-controlled clinical trial was to evaluate the safety and efficacy of using etanercept (Enbrel; Immunex Corporation) to get patients with WG into remission and maintain that remission.

All patients in this multicenter trial had active disease at the beginning of the study and most patients went into remission. Checkups occurred every three months. “We assessed their disease activity at defined time intervals, in terms of how active their vasculitis was or whether they were in remission, and we also collected information at every visit regarding the patient’s physical and mental health,” Dr. Spiera said. Investigators measured disease activity using the Birmingham Vasculitis Activity Score for Wegener’s Granulomatosis, a validated tool. At every visit, patients also filled out the Short Form 36 Health Survey, which includes a physical and mental component. Summary scores for each component are measured on a scale of 0 to 100, with 100 being the healthiest.

For their retrospective analysis of WGET, HSS investigators reviewed records of all patients who had a sustained remission of at least six months (143 patients). They then reviewed data from all checkups after the time of sustained remission to assess the relationship between flare status and the physical and mental health scores from the previous visit. They found that patients were 19 percent more likely to experience a disease flare if they had a five point lower mental health score (P<0.01) at the checkup immediately prior to the flare. The physical component score did not predict an activation of the disease.

“If you looked at patients who were in remission for six months or longer and assessed their mental health as captured by this mental health score, those with a lower mental health score at a given point in time would be more likely to be flaring at the next visit, within three months,” Dr. Spiera said. “This is the first time that as an independent variable, stress seemed to predict a greater likelihood of flaring.”

The study suggests that doctors need to be attentive to a patient’s psychological state and be proactive about interventions to help them manage stress. “There are a lot of things that can be done proactively in stress management on the patient’s side outside of seeing a psychiatrist,” Dr. Spiera said. For example, exercise and yoga have been shown to be effective stress relievers.

The HSS researchers next hope to prospectively examine the association between psychological state and flares in upcoming trials of vasculitis and other autoimmune diseases. Some investigators have hypothesized that stress-related hormones lead to immune dysregulation, but research is needed to tease out the mechanisms.

“Going forward, we can even think of trials where you would take patients who have declined in their mental component score and randomize half of them to receive some sort of stress management program and half of them not to receive it, to see if it changes their outcomes,” Dr. Spiera said.

Wegener’s granulomatosis was recently renamed granulomatosis with polyangiitis. The rare disease, in which inflamed blood vessels interfere with blood circulation, mainly affects vessels in the nose, sinuses, ears, lungs and kidneys, although other areas may be involved. It is most common in middle-aged adults.

Other authors of the study are Morgana Davids and Huong Do at HSS; Gunnar Tomasson, M.D., Boston University School of Medicine; John Davis Jr., M.D., MPH, Genentech; Gary Hoffman, M.D., Cleveland Clinic; W. Joseph McCune, M.D., University of Michigan; Ulrich Specks, M.D., Mayo Clinic; E. William St Clair, M.D., Duke University Medical Center; John Stone, M.D., MPH, Massachusetts General Hospital; and Peter Merkel, M.D., Ph.D., Boston University.

Possible first new treatment for ANCA-associated vasculitis in 40 years

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Results of an Immune Tolerance Network (ITN) clinical trial may provide doctors with another weapon in the treatment of ANCA-associated vasculitis, which has seen no new approved treatments in 40 years. The study, published in the New England Journal of Medicine, showed that the drug rituximab provides the same treatment benefits as the existing standard of care, and appears to offer additional benefits to those suffering relapses.

ANCA-associated vasculitis comprises a group of autoimmune diseases in which affected individuals make antibodies that attack their own cells – called neutrophils – causing inflammation in small- to medium sized blood vessels. This leads to subsequent organ damage, particularly in the airways, lungs and kidneys. Wegener’s granulomatosis and microscopic polyangitis are the most common forms of the disease, the former affecting 3 of every 100,000 people in the United States and accounting for approximately 1500 hospitalizations each year.

The current standard of care for these conditions is a cyclophosphamide-based regimen that has been used since the 1970s. Although this regimen has been successful in inducing remission in the majority of cases, its immunosuppressive nature puts patients at risk of serious infection and cancer. Furthermore, many patients experience relapses after the therapy is discontinued.

The current study, led by Dr. John Stone of Massachusetts General Hospital and Dr. Ulrich Specks of the Mayo Clinic aimed to establish whether rituximab, a manmade antibody currently approved for the treatment of non-hodgkins lymphoma and moderate-to-severe rheumatoid arthritis (marketed under the trade name ‘Rituxan’ by Genentech, Inc) could be just as effective.

The ITN study concludes that rituximab is not inferior to standard therapy for ANCA-associated vasculitis. However, in patients with relapsing disease, who were previously treated with cyclophosphamide at the start of the study (as opposed to those newly diagnosed), 67 percent of those in the rituximab group had no disease activity were able to discontinue all steroid use after therapy, compared with only 42 percent in the cyclophosphamide group.

“Although the two therapy regimens were equally effective in reducing patients’ disease activity, our results indicate that rituximab is superior to cyclophosphamide in inducing remission for patients experiencing a disease flare,” comments Dr. Specks.

Coupled with the fact that participants in the rituximab group received intravenous therapy once a week for 1 month–compared with 3 to 6 months of daily cyclophosphamide therapy followed by daily AZA—the observation that rituximab offers similar benefits, if not greater, for patients with ANCA-associated vasculitis is a significant advance in treatment. Investigators also did not observe any major differences in the side effects in patients from the two treatment groups.

According to Drs. Stone and Specks, ITN investigators plan to follow the participants until 18 months post treatment to determine if patients who received rituximab will relapse and to evaluate the long-term safety of this regimen.

The study was conducted by the Immune Tolerance Network and was sponsored by the National Institute of Allergy and Infectious Diseases.

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