The Connection Between Lupus and Vasculitis

Lupus (systemic lupus erythematosus) is a condition in which antibodies, immune proteins that normally protect your body from foreign substances like bacteria, attack your own tissues. When these proteins turn in this way they are called autoantibodies. They can attack nearly any tissues of the body, including blood vessels. This can cause vasculitis, or inflammation of the blood vessels.

Vasculitis can affect any type of blood vessel, big or small. This includes arteries, which carry blood from the heart to your tissues. Veins that return deoxygenated blood from the tissues to the heart can also be affected. That includes capillaries—the smallest of blood vessels through which the exchange of oxygen and carbon dioxide, as well as nutrients, takes place.

Roughly 11% to 36% of people with lupus will develop vasculitis in some tissues and to some degree.

Symptoms of Lupus-Related Vasculitis

Typical symptoms of vasculitis should be familiar to people with lupus. Fever, joint pain, fatigue, and confusion are just a few symptoms that the two conditions share.

Specific vasculitis signs and symptoms experienced often depend on what organ tissues affected blood vessels serve and the severity of the inflammation. Vasculitis affects some blood vessels in the body much more than others. For example, the kidneys are often affected in lupus, but vasculitis of the blood vessels in the kidneys is uncommon.

The Skin

Vasculitis involving the blood vessels of the skin may result in:

• Red or purple dots, often numerous, on the legs: This occurs when tiny blood vessels affected by vasculitis become weakened and break.
• Larger spots that look like large bruises
• Hives (uncommon)
• Itchy lumpy rash (uncommon)
• Painful or tender lumps: This may occur when somewhat larger blood vessels stretch and become swollen beneath the skin.

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Joints

Involvement of the blood vessels of the joints is very common and may include:

• Pain and aching due to inflammation
• Swelling and heat sensation

The Brain

Vasculitis affecting the central nervous system is perhaps the most serious complication related to lupus. It affects less than 7% of people with the disease.

Vasculitis in the brain may result in a wide variety of symptoms including:

• Headaches
• Behavioral disturbances
• Confusion
• Seizures
• Strokes

While central nervous system symptoms in lupus can be related to vasculitis, they can also be the direct result of autoantibodies acting against other tissues in the brain.

The Heart

Symptoms related to vasculitis in the heart from lupus are uncommon, but may include chest heaviness, especially that which comes on with exertion and is relieved by rest.

The Lungs

Vasculitis of the lungs can cause fever, cough, and chest X-ray findings similar to pneumonia. It may also result in lung tissue scarring (fibrosis) and chronic shortness of breath.

The Eyes

Vasculitis in the eyes that is related to lupus is usually associated with the small blood vessels in the retina. This does not always cause symptoms, but when it does they may include:

• Blurry vision which comes on suddenly and persists
• Vision loss
  

Complications

Vasculitis can be a serious condition in a few different ways. Inflammation of the blood vessel can cause it become narrowed and result in a decreased flow of blood to the tissues the blood vessel serves. Sometimes blood vessels can close off completely.

Other times, inflammation can cause blood vessels to stretch and weaken. The resulting aneurysms may then burst, causing internal bleeding and tissue damage.

Serious complications can include:

• Mesenteric vasculitis: When the mesentery (the "blanket" of connective tissue that surrounds the abdominal organs) is affected, damage to the intestine may occur. This can be thought of as an "intestinal stroke."
• Pulmonary (alveolar) hemorrhage: Involvement of larger blood vessels in the lungs can result in hemorrhage into the air sacs (alveoli).
• Mononeuritis multiplex: This is inflammation of two or more nerves that often causes deep, achy pain, and symptoms related to the affected nerves, such as foot drop (muscle weakness that makes it hard to lift the front part of your foot).
  

Other Possible Causes

Lupus is but one cause of vasculitis.

It may also occur as a result of an allergic reaction in the vessel walls or due to infection of the blood vessel walls.

Diagnosis

The diagnosis of vasculitis related to lupus can be challenging, particularly because the same symptoms that occur due to the direct effects of autoantibodies on tissues in lupus may also occur because of vasculitis itself.

A healthcare professional will review your medical history and conduct a physical exam.

Tests including a complete blood count, blood tests that look for non-specific signs of inflammation, a kidney function test, and liver function tests are also done.

Scans of the heart and brain (such as an echocardiogram or MRI angiography) may reveal aneurysms related to lupus. These lab and imaging studies are evaluated in conjunction with a careful history and physical exam to come up with the diagnosis of vasculitis.

Sometimes a biopsy is done to determine whether tissue damage is related to vasculitis.

Treatment

Most forms of vasculitis are treatable if detected before substantial organ damage has occurred.

Corticosteroids and other immunosuppressive drugs are most often used to reduce the inflammation of blood vessels. Sometimes, when steroid medications are ineffective, medications such as those used for people with leukemia and lymphomas may be helpful. One such example is Rituxan (rituximab).

Prognosis

Most of the time, making the diagnosis and beginning treatment can reduce the inflammation in blood vessels associated with vasculitis. At times, however, the condition may result in serious complications, such as those related to the formation of aneurysms.

While treatment can be effective, vasculitis is a chronic issue in which controlling symptoms, rather than curing the condition, is the approach to management.

A Word From Verywell

Vasculitis related to lupus can result in a wide array of symptoms, as well as result in serious complications related to blood vessel narrowing and aneurysms. The diagnosis can be difficult and is usually made based on a combination of findings.

While vasculitis is potentially very serious, immunosuppressive medications can frequently reduce the inflammation and may help to prevent some of the possible complications.

Since early medical care can make a difference with lupus-related vasculitis, having a good relationship with your healthcare provider and being your own advocate in your care can make a difference.