What is vasculitis?
Vasculitis is a rare disease that causes the immune system to mistakenly attack healthy blood vessels, leading to inflammation and swelling. This can make the vessel walls thicken and narrow, reducing the amount of blood and oxygen delivered to vital organs. In some cases, a blood vessel will weaken, forming a dangerous bulge or aneurysm. Any of the body’s blood vessels can be affected, including arteries, veins, and capillaries.
Penn Medicine is a national leader in autoimmune disease care and treating vasculitis and related disorders.
Types of vasculitis
All types of vasculitis cause inflammation of the blood vessels, but the symptoms and treatments can vary widely depending on which blood vessels and organs are affected. Some types of vasculitis can affect blood vessels of any size, while others are limited to just one part of the body, like the skin or brain.
Large vessel vasculitis affects the body’s biggest arteries, primarily the aorta and its major branches.
Medium vessel vasculitis involves medium-sized arteries, often those that carry blood to major organs.
Small vessel vasculitis affects tiny blood vessels, often in the skin, kidneys, or nerves. It’s linked to certain immune system reactions.
Single-organ vasculitis (SOV) is a type of blood vessel inflammation that affects only one organ or part of the body. Unlike other forms of vasculitis that involve many organs, SOV stays limited to just one area.
- Central nervous system (CNS) vasculitis attacks vessels in the brain or spinal cord.
- Cutaneous vasculitis attacks vessels in and under the skin.
Variable vessel vasculitis can affect blood vessels of any size throughout the body. Some related conditions, like IgG4-related disease, are included in this category because they can affect blood vessels of different sizes.
- Behçet’s disease commonly affects the mouth, eyes, and genitals.
- Cogan’s syndrome often inflames vessels of the eye and inner ear.
Symptoms of vasculitis
Vasculitis symptoms can vary based on the type and which organs are affected. Some people have only mild signs, while others may develop more serious problems. A vasculitis rash, which often appears as red or purple spots on the skin, is a common clue that blood vessels are inflamed. Other common signs include:
- Fever
- Fatigue
- Weight loss
- Muscle or joint pain
- Headaches
- Numbness, tingling, or weakness
- Shortness of breath or coughing up blood (if the lungs are affected)
- Stomach pain (if the digestive tract is involved)
- Vision problems (if the eyes are involved)
What causes vasculitis?
The exact cause of vasculitis isn’t always known. In some cases, it may be triggered by an autoimmune disorder like lupus, rheumatoid arthritis, or scleroderma. It can also happen after certain infections, such as hepatitis B or hepatitis C, or as a reaction to some medications. In rare cases, blood cancers or inherited genetic traits may play a role.
Diagnosing vasculitis
Diagnosing vasculitis can be tricky because its symptoms often look like those of other diseases. Doctors use blood and urine tests, imaging, and exams to look for signs of inflammation, check how organs are working, and rule out other possible causes. Taking a small sample of affected tissue (biopsy) is often the most reliable way to confirm the diagnosis.
How to treat vasculitis
Treatment for vasculitis depends on the type, how serious it is, and which organs are affected. Most people are treated with medications that lower inflammation and calm the immune system, such as corticosteroids and other immunosuppressive drugs. Supportive care is also important, including physical therapy, pain relief, and regular checkups, to help manage symptoms and improve quality of life. In more serious cases, doctors may use stronger treatments to control the disease and protect organ function.
Working across specialties to treat vasculitis
Penn Medicine is recognized nationally for excellence in treating complex vascular and autoimmune diseases. You’ll benefit from our team-based approach, where experts in rheumatology, radiology, pulmonology, ophthalmology, and other fields collaborate to devise a comprehensive care plan that’s right for you.
We offer:
- Advanced lab tests, imaging, and other specialized diagnostic tools and therapies
- Coordinated specialty care to address the wide-ranging impacts of this challenging condition
- A strong focus on research and clinical trials to bring you new and better treatments