Vasculitis

Vasculitis, sometimes called angiitis or arteritis, is an umbrella term for more than a dozen conditions, all of which involve inflammation of the blood vessels. These illnesses are rare in childhood, affecting roughly 20 in 100,000 youngsters under the age of 17. As a result, you may not have heard of vasculitis before your child was diagnosed, so it helps to first understand how the disease works.

The body is filled with an estimated 60,000 miles of blood vessels — elastic tubes that can range in size from about an inch wide (the aorta) to many times thinner than a human hair (capillary). These vessels deliver oxygen-rich blood and nutrients throughout the body and remove waste products like carbon dioxide. In vasculitis, something causes the immune system — the network of organs and cells that defends the body against outside invaders, like viruses — to mistakenly attack certain blood vessels.

This autoimmune attack (“auto” means “self”) causes the blood vessel wall to become swollen and irritated, called inflammation. Inflamed blood vessels can become narrower or even close off completely. In rare cases, they may stretch and weaken so much that they develop a bulge (aneurysm) and possibly tear open (rupture). These changes in the vessels can diminish or cut off blood flow to tissues and organs, and lead to a number of serious complications.

Remember, though, that there are many different types of vasculitis. Doctors distinguish these illnesses from one another by looking at such things as what kinds of blood vessels are affected, which organs are involved, and what kinds of symptoms are present.

The two most common forms of vasculitis in children are:

• Henoch-Schönlein purpura (HSP), which affects small blood vessels in the skin, causing a distinctive rash called purpura. It also affects small vessels in the intestines and often the kidneys. About half the children with HSP are younger than 5, although kidney problems are more likely to be severe in older children.
• Kawasaki disease, which affects small- and medium-sized vessels throughout the body, and often the coronary arteries (blood vessels around the heart). Like HSP, Kawasaki disease tends to occur in children younger than 5.

Other forms of vasculitis are far more common in adults, but can affect some children, too. These illnesses include:

• Eosinophilic granulomatosis with polyangiitis (EGPA) affects small-/medium-sized blood vessels in many parts of the body, particularly the lungs.
• Microscopic polyangiitis usually affects small blood vessels in the kidneys, central nervous system, and skin.
• Primary angiitis of the central nervous system (PACNS) affects small-/medium-sized blood vessels of the brain and spinal cord; sometimes called CNS vasculitis.
• Polyarteritis nodosa (PAN) affects small-/medium-sized blood vessels throughout body, including the skin, kidneys, and peripheral nerves (nerves that travel from the spinal cord to the rest of the body).
• Takayasu arteritis affects large blood vessels, especially the aorta (the major blood vessel that carries blood from the heart to the rest of the body).
• Granulomatosis with polyangiitis (GPA) usually affects small-/medium-sized blood vessels in the lungs, kidneys, and skin.

While vasculitis can’t be prevented or cured, it can go into remission, meaning the disease is not active, and its signs and symptoms go away. With early diagnosis and the right treatment, the vast majority of children with vasculitis can achieve remission and go on to lead full and normal lives.

There’s no single set of symptoms for vasculitis, since different forms of this disease can affect different parts of the body. Broadly speaking, though, most children with vasculitis have symptoms of general illness, including:

• Fatigue
• Fever
• Weight loss
• Loss of appetite
• Aches all over

Symptoms that may indicate vasculitis is affecting a particular part of the body include:

• Purplish rashes that don’t blanch (fade) when you press on them
• Shortness of breath and/or chest pain
• Abdominal pain and/or bloating
• Joint and muscle pain
• Nerve problems (numbness, tingling)
• Blurring or loss of vision
• Headaches
• Blood in the urine

Because vasculitis attacks the blood vessels, it also has the potential to affect the organs and tissues that depend on those blood vessels for oxygen and other nutrients. This can cause complications ranging from relatively mild (skin ulcers) to very serious (organ failure).

The complications your child might face will depend on what type of vasculitis he has and which organs are involved. For instance:

• Henoch-Schönlein purpura often targets blood vessels in the kidneys, raising the risk of kidney disease (nephritis).
• Kawasaki disease tends to involve blood vessels around the heart, which in rare instances can cause such things as abnormal heart rhythm (arrhythmia) or even heart attack.

Infection is also a potential complication for vasculitis patients — partly because the immune system is already malfunctioning, but also because many vasculitis medications work by actually suppressing the immune system (which further lowers the body’s defenses against invaders like bacteria and viruses).

Your child’s doctor will be able to discuss specific complications with you in detail, as well as how they can be prevented or treated.

Vasculitis belongs to the family of autoimmune diseases, in which the body’s immune system is mistakenly attacking its own healthy cells and tissues. Researchers don’t yet understand exactly why this happens, but believe there is a combination of genetic and environmental factors at work.

Among the factors that seem to trigger certain kinds of vasculitis are infections, like hepatitis B and streptococcus; other autoimmune diseases, such as lupus and scleroderma; and cancers that affect the blood cells, like leukemia and lymphoma. However, many children who have these illnesses do not develop vasculitis — which underscores the fact that its causes are complex and, so far, not fully known.

Because vasculitis can affect blood vessels in virtually any part of the body, its signs and symptoms vary greatly — from rashes and muscle cramps to chest pain. But in general, to make a diagnosis of vasculitis, your child’s doctor will be looking for:

• Signs of general inflammation, including fatigue, low levels of red blood cells (anemia), and elevated blood proteins
• Symptoms of decreased or obstructed blood flow to specific parts of the body
• Direct evidence of blood vessel inflammation through imaging tests or biopsy

Since there’s no single test to show what kind of vasculitis your child has, your child’s doctor will gather information from a thorough physical exam and medical history plus a variety of diagnostic tools, such as:

• Blood tests to look for signs of inflammation in the body (namely, abnormal levels of blood cells or antibodies), including:
• Erythrocyte sedimentation rate (sed rate)
• C-reactive protein (CRP) test
• Complete blood cell count (CBC)
• Anti-neutrophil cytoplasmic antibodies (ANCA) test
• Urine tests to check for the presence of red blood cells or high levels of proteins, which may indicate that vasculitis is affecting the kidneys
• Imaging tests to examine the blood vessels and other internal organs. For example:
• A chest X-ray may show whether vasculitis is affecting the lungs or large arteries.
• Abdominal ultrasound may show whether vasculitis is affecting the intestines or other abdominal organs.
• Computerized tomography (CT) and/or magnetic resonance imaging (MRI) may show whether vasculitis is affecting the brain.
• An angiogram (a kind of X-ray in which special dye is injected into an artery or vein) may show how vasculitis is affecting the blood vessels themselves.
• Biopsy (the surgical removal of a tissue sample) to check for direct evidence of inflammation in a blood vessel or other organ

After making a diagnosis, your child’s doctor will also use many of these same tests to monitor your child’s condition. But remember: As a parent, you have a vital part to play, too, by keeping the doctor fully up to date on any new symptoms or changes in your child’s health.

In some children, certain forms of vasculitis will improve on their own — for instance, Henoch-Schönlein purpura may run its course in four to six weeks with no treatment beyond bed rest, plenty of fluids, and occasionally nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. But other children will need medications to control their vasculitis and head off or treat serious complications.

Medications commonly used to treat vasculitis include:

• Corticosteroids — often prednisone — are not the same as the anabolic steroids that athletes sometimes take. These are extremely powerful, fast-acting drugs that fight inflammation by suppressing the entire immune system. However, doctors work to phase them out as soon as possible because of their potential side effects, which can include high blood pressure and reduced bone density.
• Steroid-sparing immunosuppressants offer many of the benefits of corticosteroids — and sometimes with fewer side effects — but take longer to work. The most powerful of this group is cyclophosphamide (Cytoxan); others include azathioprine (Imuran); methotrexate;and mycophenolate mofetil, or MMF (CellCept).
• Biologics are a relatively new class of steroid-sparing therapies based on compounds made by living cells. Instead of suppressing the entire immune system, biologics are more like smart bombs — they only target certain parts of it. Biologics now being used for vasculitis include tumor necrosis factor (TNF) blockers such as infliximab (Remicade). Side effects vary by medication, but are generally milder than with other kinds of steroid-sparing therapies.

Children with some kinds of vasculitis — particularly Kawasaki disease — may also benefit from IVIg (intravenous immunoglobulin). This is a blood product made up of healthful antibodies that is delivered by IV, and can help get the immune system back on track without suppressing its normal function.

In rare instances, children with vasculitis will develop complications that require surgery — for instance, if an abnormal bulge (aneurysm) develops in a blood vessel wall, doctors may need to operate to keep it from rupturing.

What the future may hold for your child will depend a good deal on what kind of vasculitis he has. Kawasaki disease, for instance, tends to be what doctors call “self-limiting” — the illness usually runs its course in a month or two and does not recur. However, there is a lot of variation even within the individual types of vasculitis, depending on how severe the disease is and what organs are involved. Looking at the example of Kawasaki again, if there is serious inflammation in the coronary arteries, it may cause permanent heart damage.

In all kinds of vasculitis, doctors aim to quench the inflammation and achieve remission — that is, the disease becomes completely “quiet,” with no signs or symptoms. Once the vasculitis is under control, they may then cautiously withdraw medications with the hope that the disease will stay in remission.

Your child’s doctor will be the best source of information on the likely long-term outlook for your child. However, if diagnosed and treated before any serious complications have occurred, the vast majority of children with vasculitis will achieve remission and go on to lead full and normal lives.

Boston Children's Hospital successfully treats many kinds of childhood vasculitis. Our rheumatologists, the specialists with the most experience in diagnosing and treating vasculitis, make up one of the largest pediatric rheumatology departments in the U.S., seeing more than 4,000 outpatients and almost 1,000 inpatients every year.

Our Samara Jan Turkel Clinical Center for Pediatric Autoimmune Diseases brings together pediatric rheumatologists and consulting specialists from across the hospital to offer comprehensive, coordinated care for children with vasculitis.

We’ve established unique collaborations between departments, such as the Dermatology-Rheumatology Center, which unites rheumatologists and dermatologists in caring for children with vasculitis involving the skin. Another example is the Multiple Manifestations of Autoimmune Disease Clinic, where rheumatologists and immunologists work together to help children with many autoimmune problems, including vasculitis for which a single diagnosis doesn’t fit.