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Lupus and Vasculitis: What’s the Link?

Medically Reviewed by Melinda Ratini, DO, MS on March 10, 2022

Lupus can affect multiple parts of your body, including your blood vessels. When they become inflamed, that’s called vasculitis.

Vasculitis isn’t the most common complication of lupus. But anyone with the autoimmune condition can get it. Symptoms are generally mild. You may feel tired or flu-like.

But vasculitis can be life-threatening for some people. It depends on which organs are affected and how serious the tissue damage is. Work with your doctor to diagnose and treat any blood vessel inflammation early.

How Does Lupus Cause Vasculitis?

One function of the immune system is to make antibodies, or germ fighters, to attach to antigens -- toxins or foreign substances. Antibodies bind to antigens to create something called an immune complex. Certain parts of your immune system, or defenses, then spot and destroy these complexes. That’s how we fight off infections.

But when you have lupus, your body can’t tell the difference between what’s healthy and what’s harmful. So you form other kinds of immune complexes made of “autoantibodies” and “self-antigens.”

Lupus-related immune complexes can float around in your blood and stick to your blood vessel walls. White blood cells can destroy these immune complexes the same way they would viruses or bacteria. But that can eat away at the walls of your blood vessels or surrounding tissue, causing them to burst.

Who Gets Vasculitis?

Studies show around 11% to 36% of people with lupus might get vasculitis. But it’s hard to predict exactly who’ll get blood vessel inflammation.

Here are some factors that raise your chances:

Your lupus isn’t well-controlled. Vasculitis is more common during a flare.

You take certain medication. Some drugs might trigger changes in your blood vessels. If this is what’s causing your vasculitis, the inflammation will likely go away when you quit the medication. Some drugs not typically used for lupus can be linked to vasculitis and include:

Talk to your doctor before making any changes to your treatment plan.

You have anti-neutrophil cytoplasmic antibodies (ANCA). These can lead to inflammation in your kidneys. It’s not common, but you can have lupus along with ANCA. Your doctor can run a blood test to find out.

You have other medical conditions. Lupus is only one health issue that can lead to vasculitis. Some others include:

How Does Vasculitis Damage Your Body?

Lupus vasculitis usually affects your small blood vessels, or capillaries. Less commonly, you can get inflammation in medium and large blood vessels -- your veins and arteries.

Here’s how vasculitis might look in different parts of your body.

Skin. Lupus vasculitis most often occurs in the small blood vessels of the skin. You might hear your doctor call this cutaneous vasculitis. There’s some evidence that your odds of getting it are higher if you’re Black and have lupus.

Symptoms may come and go, but you might notice:

  • Red or purple spots, usually on your legs
  • Dark spots in your fingertips, toes
  • Broken blood vessels in your cuticles
  • Areas that look bruised
  • Itchy bumps or rashes
  • Tender lumps under the skin

Intestinal system. Rarely, you can get inflammation in the medium-to-large blood vessels that go to your intestines. That’s called lupus mesenteric vasculitis (LMV). Symptoms include mild to severe belly pain that comes on suddenly. LMV can be life-threatening.

Joints and nerves. Your joints may swell and hurt. Without enough blood flow, you may have numbness or tingling in your arms, legs, fingers, or toes. You could lose feeling or have weakness in your feet or hands.

Eyes. Vasculitis can affect the small blood vessels in your eyes. You might not have any symptoms. But call 911 if you have sudden blurry vision or a loss of sight.

Other organs. It doesn’t happen a lot, but it’s possible for vasculitis to affect your heart, lungs, or kidneys. You may not have any symptoms. But you could have:

  • A heavy feeling in your chest when you’re active (heart vasculitis)
  • Shortness of breath, fever, or cough (lung vasculitis)
  • High blood pressure (kidney vasculitis)

Get emergency medical help right away if you have chest pressure, or new or worsening shortness of breath.

Brain. Rarely, you can get inflammation in the big or little blood vessels in your brain or spine. That’s called cerebral vasculitis or central nervous system vasculitis. Your symptoms can be mild or life-threatening.

Get help right away if you have:

  • Serious headaches that don’t go away
  • Dizziness
  • Memory or vision problems
  • Weakness or weird sensations
  • Seizures

How Do You Diagnose Vasculitis?

Your doctor will give you a physical exam and go over your health history. They’ll get a list of your current symptoms and check for inflammation from other health conditions. Some can look like vasculitis.

You might need one or more of the following:

Blood tests. Your doctor will look for signs of inflammation and certain antibodies. They might check for:

  • High levels of white blood cells
  • High platelet count
  • Anemia
  • A positive ANCA test
  • High levels of C-reactive protein

Urine test. They’ll check for kidney damage.

Tissue biopsy. This is a kind of surgery. It’s the best way to diagnose vasculitis. Your doctor will cut out a sample of the affected area, such as your skin or kidney. A health professional will look at that tissue under a microscope to check for inflammation.

Other tests. You may need X-rays of your blood vessels (angiography) or other special tests to check for vasculitis in your heart, lungs, brain, or other organs. Your doctor will let you know what’s best for you.

How Do You Treat Vasculitis?

You may not need to do anything if you have only a few spots on your skin. But it’s important to get the right treatment early on. The goal is to get you into remission.

Your doctor will consider a few things when deciding your care, including:

  • What type of blood vessels are involved
  • How serious your vasculitis is
  • Your general health
  • How you’ve reacted to medication in the past

Treatment for vasculitis includes:

Corticosteroids. These drugs slow down your immune system to quickly stop inflammation and cell damage. They’re often the first kind of medicine used for vasculitis. You might be on them for a long time, but they come with side effects. Your doctor will go over the pros and cons of short- and long-term treatment.

Corticosteroids used for vasculitis include:

  • Prednisone
  • Prednisolone
  • Methylprednisolone

Cytotoxic drugs. These kill the cells that cause blood vessel inflammation. They might be used alongside or instead of steroid medication.

Examples of cytotoxic drugs used for vasculitis include:

Biologics. These are drugs that change how your immune system works. A biologic called rituximab (Rituxan, Ruxience, Truxima) is approved to treat ANCA-associated vasculitis. It might be a good choice if your vasculitis comes and goes a lot.

Plasma exchange. Also called plasmapheresis, a machine can “clean” part of your blood to get rid of cells that cause inflammation. Your doctor might suggest this or other therapies if common treatments don’t work and your symptoms are really serious.

Treatment works really well for most people. You may not have any serious or long-term symptoms. But in some cases, vasculitis can get bad really fast. You may not be able to prevent or undo some of the damage.

Keep your doctor in the loop about your symptoms. They’ll help you manage your lupus and vasculitis.

Show Sources

SOURCES:

Fotios Koumpouras, MD, FACR, assistant professor of medicine (rheumatology), Yale School of Medicine; director, Yale Lupus Program.

Lupus Foundation of America: “Vasculitis and lupus.”

Current Rheumatology Reports: “Lupus Vasculitis,” “Vasculitis in systemic lupus erythematosus.” 

Hospital for Special Surgery (HSS): “Lupus, Vasculitis and Blocked Blood Vessels.”

Biomedicines: “Lupus Vasculitis: An Overview.”

Frontiers in Immunology: “Mechanisms of Autoantibody Production in Systemic Lupus Erythematosus.”

The Journal of Medicine (Netherlands): “Drug-induced vasculitis: a clinical pathological review.”

Lupus Science & Medicine: “Cutaneous vasculitis in SLE.”

National Heart, Lung, and Blood Institute (NIH): “Vasculitis.”

Lupus: “Cerebral large vessel vasculitis in systemic lupus erythematosus.”

Frontiers in Pediatrics: “Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus.”

Mayo Clinic: “Vasculitis.”

JAMA Dermatology: “Cutaneous Vasculitis in Systemic Lupus Erythematosus.”

JAAD Case Reports: “Systemic lupus erythematosus and antineutrophil cytoplasmic antibody — associated vasculitis: An emerging overlap syndrome with cutaneous manifestations.”

Nephrology Dialysis Transplantation: “Lupus nephritis and ANCA-associated vasculitis: towards precision medicine?”

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