Vasculitis

What is vasculitis?

Vasculitis is an umbrella term for a group of autoimmune diseases that cause inflammation of the blood vessel walls throughout the body. In autoimmune diseases, the body’s immune system mistakenly attacks itself, causing inflammation. Vasculitis causes inflammation in the walls of the veins, arteries, and tiny capillaries that carry blood throughout the body. When blood vessels become swollen or irritated, it becomes more difficult for the blood to travel and deliver oxygen and nutrients to organs in the body.

Physicians at the Pediatric Rheumatology Program diagnose and treat all forms of childhood vasculitis. Our Kawasaki Disease Program provides coordinated care and expertise for children with Kawasaki disease.

The most common forms of vasculitis in children are:

• Henoch-Schonlein purpurn
• Kawasaki disease

Less common forms of vasculitis in children include:

• Takayasu’s arteritis
• Polyarteritis nodosa
• ANCA-associated vasculitis
• Churn-Strauss syndrome
• Behcet’s disease
• Primary CNS vasculitis

Vasculitis may be a chronic, or ongoing, condition. When it is diagnosed and treated early, symptoms for many children go into remission (no symptoms) and often do not recur. The goal of treatment is to achieve remission so that young patients can lead full, productive lives.

What are the symptoms of vasculitis?

Vasculitis can affect blood vessels anywhere in the body, and symptoms vary widely. Your child’s symptoms will depend on the type and severity of vasculitis. General symptoms include:

• Unexplained fever
• Weight loss
• Skin rash
• Joint pain or swelling
• Headaches
• High blood pressure

Other, more serious symptoms may include:

• Lung problems, including hemorrhage
• Nerve problems
• Blood in the urine
• Loss of vision

How is vasculitis diagnosed?

Early and expert diagnosis is important for children with vasculitis, because the disease can quickly become very serious. If vasculitis is suspected, our rheumatologists will conduct a full medical exam and schedule a series of tests to determine whether and what type of vasculitis is causing the symptoms your child is experiencing. Tests will look for signs of inflammation and damage to organs and may include:

• Blood and urine tests
• Imaging tests
• Echocardiogram
• Lung function test
• Biopsy

How is vasculitis treated?

The goal of treatment for any form of vasculitis is remission, or absence of symptoms. For most children who are diagnosed and treated early—before any damage to organs has occurred—symptoms can go into remission and may not recur.

Physicians at the Pediatric Rheumatology Program work closely with families to create comprehensive treatment and follow up plans for children with all types of vasculitis.

Specific treatments will depend on the type of vasculitis and the severity of the disease. Some forms of vasculitis run their course with very little treatment other than rest and nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen to ease discomfort. Medications such as corticosteroids, immunosuppressants, and biologics may be part of your child’s treatment plan.

Rarely, surgery is necessary for complications caused by vasculitis.

Many children with vasculitis require long-term follow up care from a pediatric rheumatologist. Follow up care may involve some of the tests used to diagnose the disease. If remission is achieved, your child’s doctor may withdraw medications slowly.