A rare-but-serious family of diseases you may never have heard of. When there is swelling or inflammation in the body’s blood vessels, the cause may be vasculitis. That’s an umbrella term for more than 30 uncommon conditions—some of them life-threatening.
What Is Vasculitis?
It is a condition that involves inflammation in the blood vessels. It can result in poor blood flow to the body’s organs and tissues. Vasculitis occurs when your immune system attacks your blood vessels. This can happen due to infection, reaction to a medicine, or another disease or condition.
Blood vessels include:
- Arteries that carry blood from your heart to your body’s organs
- Veins that carry blood from your organs and limbs back to your heart
- Capillaries that connect your small arteries and veins
“There are well over 30 different types of vasculitis,” notes Dr. Peter Grayson. He is founder and principal investigator of the Vasculitis Translational Research Program (VTRP) at the NIH’s National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). “We group them predominantly based on the size of the blood vessels that are affected.”
Vasculitis is believed to be an autoimmune disease. This is where the immune system is dysfunctional or confused. The immune system, rather than fighting off infections, actually starts to attack blood vessels.
“There is no single test for diagnosing vasculitis,” Dr. Grayson says. “Diagnosing comes down to pattern recognition. But even within individual conditions, there are variations.”
This can make diagnosis a challenge for health care providers.
“Thirty years ago, many of these diseases were fatal. Today, we try to find out what drug will be right for each person,” Dr. Grayson says. “Treatment often works well if it’s started early. In most cases, vasculitis can go into remission with treatment. This means the condition isn’t active, but it can come back, or ‘flare,’ at any time.”
Sometimes vasculitis is chronic, or ongoing, and never goes into remission. Long-term treatment with medicines often can control vasculitis.
Much is still unknown about vasculitis. But researchers, including Dr. Grayson and his team, continue to learn more about the condition and its various types, causes, and treatments.
Although most patients with vasculitis achieve remission with treatment, many experience at least one relapse. A major focus of Dr. Grayson and his team is to identify when a relapse might occur. Markers in the blood and tissues can guide treatment decisions for people with the disease.
The VTRP also collaborates with the NIH-supported Vasculitis Clinical Research Consortium. It is a network of academic medical centers, patient support organizations, and clinical research resources. Fifteen participating clinical centers throughout the United States and Canada currently conduct studies.
NIH-supported researchers are working to identify the genetic and biologic structures of the various forms of vasculitis. Their goal is to develop more targeted and less toxic therapies for people with the disease.