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.
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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.
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:
- Vasculitis is inflammation of blood vessels. The body’s immune system regulates inflammation.
- 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.
- 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.
- The cause of most forms of vasculitis is unknown.
- 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.
- 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.
- 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.