Almost half of the 5 million people living with systemic lupus erythematosus (SLE) develop vasculitis. This lupus complication causes inflammation in your blood vessels. The inflammation can affect the veins (phlebitis), the arteries (arteritis), the capillaries, or all three (systemic vasculitis). When vasculitis occurs, blood flow may be reduced, which can lead to tissue and organ damage. In people with lupus, it’s sometimes called lupus vasculitis.

“I have vasculitis in my eyes,” one MyLupusTeam member wrote. “It is very painful and when it flares, I look like someone possessed.” Another said, “I have had vasculitis twice in my roller coaster ride with SLE.”

Causes of Vasculitis

Researchers aren’t sure what causes vasculitis in people with lupus. However, they find 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 (a common virus that often doesn’t cause symptoms) or hepatitis C.

Other forms of vasculitis may develop as a side effect of medications prescribed to control lupus or its symptoms. These may include biologics (medicines made from living cells) and antiepilepsy drugs such as carbamazepine.

Granulomatosis with polyangiitis (formerly called Wegener’s granulomatosis) is another type of vasculitis. Some research has found a genetic link between this condition and autoimmune diseases like lupus. However, more research is needed to understand this connection better.

Symptoms of Vasculitis

When the blood vessel walls become inflamed, they can thicken, scar, or become weak in certain areas. These changes make it difficult for blood to flow properly. Blood circulation can slow down, limiting how much oxygen and nutrients reach the cells and causing them to stop working properly.

Depending on where in the body vasculitis develops, the condition can cause a wide range of symptoms. It may affect the skin, joints, brain, nerves, kidneys, 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 (discolored dots), especially on the legs
• Purpura (larger purple, red, or darker 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 the peripheral nervous system, leading to:

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

Heart and Lung Symptoms

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

• Pneumonialike symptoms and lung changes seen on X-ray
• Cough
• Fever
• Scarring of the lungs
• Hemoptysis (sputum mixed with blood)
• 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

Kidney-related symptoms include:

• Proteinuria (passing of protein in the urine)
• Hematuria (passing of blood in the urine)
• Urine sediments
• Sudden kidney failure

How Do You Test for Lupus Vasculitis?

Your rheumatologist or other specialist will take a complete medical history and perform a physical examination to look for symptoms. They may order blood tests to check blood cell levels and signs of inflammation. Your doctor may also order urine tests. Blood and urine tests can help determine the type of vasculitis you have or what parts of the body are affected.

Other tests to diagnose vasculitis may include:

• Biopsy — Doctors can remove a small piece of affected tissue to help diagnose the type of vasculitis you have.
• Echocardiogram and electrocardiogram — These two tests can be used to assess how vasculitis is affecting the heart. They may be ordered if you have symptoms like chest pain or pressure.
• X-ray — Chest X-rays can show if your heart or lungs are affected. If your doctor thinks it’s necessary, they’ll order a CT scan of the chest.
• Angiography — This special type of X-ray can help identify blocked arteries or veins throughout the body, including the brain.
• CT scan, MRI, and ultrasound — These scans can be used to evaluate internal organs. CT scans and MRIs can be used to check for vasculitis in the central nervous system.

Treating Vasculitis

Treating vasculitis depends on the organs involved, how severe the inflammation is, your overall health, and how you’ve responded to different treatments in the past. In mild cases, no treatment may be required. Diagnosing serious vasculitis that could harm major organs may require stronger immune-suppressing treatment. Severe vasculitis that needs treatment may be managed with the therapies listed below.

• Corticosteroids — Also called steroids, these may help reduce and manage blood vessel inflammation.
• Disease-modifying antirheumatic drugs (DMARDs) — These drugs may help calm inflammation. Biologics (a type of DMARD made from living cells) may be used especially for organ-threatening vasculitis. Compared to other DMARDs, biologics target specific parts of the immune system, often have fewer side effects, and can lead to better results.
• Plasmapheresis — This process involves taking out some of the body’s plasma and replacing it with healthy plasma to remove harmful antibodies and reduce the immune response. It’s especially important if the vasculitis is very severe and life-threatening. If someone can’t tolerate plasmapheresis, intravenous immunoglobulins may be used instead.
• Surgery — In some advanced cases, doctors may recommend procedures such as vascular bypass surgery. In this surgery, blood flow is rerouted away from damaged vessels so it moves through healthy vessels instead.

Treating vasculitis is an ongoing process that requires consistent monitoring by your rheumatology team or other health care professionals. If vasculitis develops as a complication of lupus, keeping lupus under control with the right level of immune-suppressing treatment can help manage the vasculitis more effectively.

Talk With Others Who Understand

On MyLupusTeam, the social network for people with lupus, more than 232,000 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.