What is vasculitis?

Vasculitis is an inflammation of the blood vessels. When a blood vessel becomes inflamed, it can narrow — making it more difficult for blood to get through. It can also close completely, or stretch and weaken, which can cause an aneurysm.

Vasculitis occurs when the body’s immune system mistakenly attacks one or more blood vessels. The cause of vasculitis can be an infection, medicine or another disease. In many cases, the cause is unknown.

Vasculitis can affect all types of blood vessels including:

• Veins, which carry blood to the heart from body organs
• Arteries, which carry blood from the heart to body organs
• Capillaries, which connect the small arteries and veins

Any body organ can be affected by vasculitis. The organs affected by vasculitis can help your child’s doctors determine which type of vasculitis he has.

Types of vasculitis

The most common types of vasculitis are:

• Henoch-Schönlein purpura, also known as IgA vasculitis
• Kawasaki disease

Less common forms of vasculitis in children include:

• Behçet’s syndrome
• Buerger’s disease
• Cogan’s syndrome
• Cryoglobulinemia vasculitis
• Eosinophilic granulomatosis with polyangiitis
• Giant cell arteritis
• Granulomatosis with polyangiitis, also known as Wegener’s granulomatosis
• Hypersensitivity vasculitis
• Microscopic polyangiitis
• Polyarteritis nodosa
• Polymyalgia rheumatica
• Primary angiitis of the central nervous system, also known as central nervous system vasculitis
• Takayasu’s arteritis

At The Children’s Hospital of Philadelphia, we diagnose and treat all forms of vasculitis.

Causes

Certain types of vasculitis may be triggered by infection, drugs, or other environmental triggers. Other types of vasculitis are thought to be caused by an underlying autoimmune disease.

In rare instances, vasculitis has been linked to mutations in specific genes resulting in blood vessel inflammation. However, most of the time, the cause of vasculitis is unknown.

Signs and symptoms

Doctors may suspect your child has vasculitis if the child has:

• Unexplained daily fevers (greater than 101 degrees Fahrenheit) for more than two weeks
• A disease that typically affects only adults found to be occurring in a child (example: a child with a heart attack or a child who develops a stroke)
• A skin rash with small, red or purple raised areas
• Evidence of many organ systems being damaged or injured at the same time

Symptoms of vasculitis vary from child to child and differ depending on the specific type of vasculitis causing disease. Symptoms may include:

• Severe and prolonged tiredness
• Prolonged fevers
• Muscle or joint pains
• Lack of appetite or severe weight loss
• Belly pain or bloody diarrhea
• Brown or dark-colored urine
• Numbness or weakness
• A skin rash
• Coughing up blood

Testing and diagnosis

Prompt diagnosis and treatment of vasculitis is critically important because the disorder can quickly become life-threatening or organ-threatening if not managed appropriately.

However, the disorder can be challenging to diagnose. Patients often present with a wide variety of symptoms that mimic many other disorders such as infections, drug reactions, toxic ingestions, autoimmune diseases, or other types of tissue-damaging processes.

To ensure an accurate diagnoses and proper treatment, experts recommend children be evaluated by a multidisciplinary team like the one at The Children’s Hospital of Philadelphia.

At CHOP, assessment for vasculitis begins with a thorough medical history, a physical exam of your child and a review of your child’s symptoms.

If clinicians suspect your child has vasculitis, further testing or clinical observation may be needed to confirm the diagnosis, including:

• Blood and urine tests to identify whether there is evidence of inflammation in the body and to help identify your child’s specific type of vasculitis
• An echocardiogram to determine if your child’s heart is functioning properly
• Lung function tests to determine if your child’s lungs are working properly
• Ultrasound, CT scans, MRIs, or angiography to take pictures of various parts of your child’s body, including blood vessels, to determine if they are damaged or not working properly
• A biopsy — which involves using a needle to remove a small tissue sample for examination under a microscope — may be used to look for inflammation within blood vessels.

Treatment

Treatment for vasculitis will be determined by your child's doctor based on:

• Your child's overall health and medical history
• The type of vasculitis diagnosed
• The specific organs affected
• The severity of your child’s condition
• Your child's tolerance for specific medications, procedures and therapies
• Your family’s opinion or preference

Treatments for vasculitis may include:

• Use of medications to control pain
• Supportive care to help organs that may not be functioning properly because of the vasculitis
• Specialized medications to help control inflammation

Follow-up care

Ongoing care and management for children with vasculitis will depend on the severity of disease, the involvement of major organs, and which type of vasculitis your child has.

In most cases, children with vasculitis will require ongoing disease monitoring for weeks, months and often years after the initial episode of vasculitis. Follow up care will be tailored to meet your child’s individual needs.

Patients with vasculitis may need routine follow-up care with a variety of specialists, including:

• Rheumatologists, who assess joints, muscles, bones and the immune system
• Nephrologists, who monitor kidney function
• Pulmonologists, who assess lung function
• Neurologists, who monitor brain and nerve function
• Cardiologists, who assess heart health
• Gastroenterologists, who monitor liver and intestine function
• Other medical providers depending on the organs involved and the overall disease manifestations

Outlook

The outlook for children with vasculitis varies widely. Some patients will heal completely from a bout of vasculitis, without the need for any treatment at all. Other patients will develop severe life-threatening disease, may have permanent damage to vital organs or may accumulate life-long toxicities from medications used to treat the vasculitis.

Given the complexity of this condition, it’s important to seek care at a specialized center where physicians have expertise in the diagnosis and treatment of vasculitis and its complications and can provide state-of-the-art supportive care.