An estimated 1.5 million Americans and 5 million people around the world live with lupus. As many as half of those diagnosed with systemic lupus erythematosus (SLE) develop vasculitis, a complication of the autoimmune disease where inflammation moves into the vascular system. Vasculitis results in inflammation of the blood vessels. It can affect the veins (phlebitis), the arteries (arteritis), very small blood vessels called capillaries, or all three (systemic vasculitis).

Causes of Vasculitis

Although researchers aren’t sure what causes vasculitis in people with lupus, they are finding complex connections between an overly aggressive immune system and inflammatory changes that lead to vasculitis. Some forms of vasculitis can develop in people with lupus when they contract certain viruses, such as cytomegalovirus or hepatitis C. Other forms of vasculitis may develop as a side effect of medications prescribed to control lupus or its symptoms, such as monoclonal antibodies (also known as biologics) and antiepilepsy drugs such as carbamazepine.

A 2010 study indicated a genetic link between Wegener’s granulomatosis (another type of vasculitis) and autoimmune conditions such as lupus or rheumatoid arthritis, but more research is needed to further understand this connection.

Symptoms of Vasculitis

When the blood vessel walls become inflamed, they can thicken, scar, or develop areas of weakness. All of these changes make it difficult for blood to flow properly. Circulation can slow down, which can limit how much oxygen and nutrients reach the cells and inhibit normal cell functioning.

Depending on where in the body vasculitis develops, the condition can cause a wide array of symptoms affecting the skin, joints, brain, nerves, heart, lungs, or intestines. Symptoms can range in severity from mild to life-threatening.

Skin and Joint Symptoms

When vasculitis affects the skin and joints, it can cause:

• Petechiae (small red or purple dots), especially on the legs
• Purpura (larger purple or red spots that may look like bruises)
• Hives
• Rash with itchy, painful, or tender lumps
• Ulcers on the ankles
• Necrosis (black spots of dead tissue) or gangrene on the fingers or toes
• Joints that are painful, hot, or swollen

Brain, Eye, and Nerve Symptoms

Vasculitis can develop in the central nervous system (the brain and spinal cord), eyes, or peripheral nervous system, leading to:

• Headaches
• Behavior changes
• Seizures or strokes
• Confusion
• Vision changes or loss of vision

Heart and Lung Symptoms

When vasculitis impacts the heart or lungs, it may cause symptoms like:

• Pneumonia-like symptoms and lung changes seen on X-ray
• Cough
• Fever
• Scarring of the lungs
• Shortness of breath, which may become chronic
• Rarely, angina (pain or heaviness in the chest during exertion)

Intestinal Symptoms

Vasculitis may cause problems with blood flow in the intestines, potentially leading to:

• Abdominal pain
• Bloating
• Cramping
• Blood in the stool

In severe cases, vasculitis may lead to perforation of the intestines requiring emergency surgery.

Diagnosing Vasculitis

Your rheumatologist or other specialist will check for symptoms, take a complete medical history, and perform a physical examination. They may order blood tests as well as other laboratory tests to check blood cell levels and inflammation markers. Markers might include an elevated sedimentation rate and antineutrophil cytoplasmic antibodies.

Other tests may include an electrical test of your nerve function, a biopsy of affected tissue, or an electrocardiogram of your heart. Imaging tests such as ultrasounds, X-rays, or angiography can look at blood vessels in your lungs, abdomen, and other areas. If vasculitis in the central nervous system is suspected, you may undergo a CT scan, MRI scan, or cerebral angiography (angiogram of the brain). Your doctor will make the diagnosis after reviewing all of your information and test results.

Treating Vasculitis

Treating vasculitis depends on the organs involved, the severity of the inflammation, your overall health, and how you have responded to different treatments in the past. In mild cases, no treatment may be required.

Vasculitis that is severe enough to require treatment is often initially treated with corticosteroids such as prednisone to help reduce and manage blood vessel inflammation.

If you have severe disease that does not respond to corticosteroids or you experience unwanted side effects from medications, your doctor may prescribe immunosuppressive cytotoxic drugs to kill the cells that contribute to inflammation. These may include:

• Imuran (azathioprine)
• Cytoxan (cyclophosphamide)
• Rituxan (rituximab)

Treating vasculitis is an ongoing process that requires consistent monitoring by your health care professional.

Talk With Others Who Understand

On MyLupusTeam, the social network for people with lupus, more than 201,000 members come together to ask questions, give advice, and share their stories with others who understand life with lupus.

Are you living with vasculitis? How has it affected you? Have you found an effective way to treat it? Share your experience in the comments below, or start a conversation by posting on your Activities page.