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. Not knowing much about what is making your child ill can be upsetting for a parent, 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:
- Churg-Strauss syndrome -- 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)
- Wegener granulomatosis -- usually affects small-/medium-sized blood vessels in the lungs, kidneys and skin
In diagnosing your child’s specific kind of vasculitis, the doctor will talk with you in detail about the illness and the outlook for your child. But the overriding treatment goal for all forms of vascuilitis is the same: to bring the harmful inflammation under control as soon as possible and to work toward disease remission.
Who is at risk?
In general, vasculitis can affect people of all ages, genders, races and nationalities. However, some types of vasculitis are more likely to occur in certain groups than others. For instance:
- Henoch-Schönlein purpura occurs in children and young adults, with the primary risk group being children 2 to 11 years old. It’s nearly twice as common in boys, and tends to affect whites and Asians more than other racial groups.
- Kawasaki disease occurs almost exclusively in children: An estimated 80 to 90 percent of Kawasaki patients are younger than 5. It’s more common in boys, and affects Asian children five to 10 times as often as white children.
- Takayasu arteritis occurs primarily in young adults, though it can crop up in children as young as 1. It’s up to nine times as common in females, and tends to affect Asians more than other racial groups.
Beyond age, race and gender, some people may be at greater risk for vasculitis if they’ve had certain infections or other illnesses. For instance, in polyarteritis nodosa (which is rare in children), an estimated 30 to 50 percent of patients have had hepatitis B or C.
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.
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:
- 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
- blood in the urine
Whenever your child is sick, it’s natural to wonder whether there’s anything you as a parent could done to prevent it. But vasculitis isn’t that kind of illness -- it’s not contagious or infectious, and no diet or exercise plan can ward it off.
Researchers are still working to understand exactly what causes vasculitis. Much of that effort centers on identifying genetic and biochemical markers -- things within a person’s own body that point to whether he or she might develop vasculitis -- which could help pave the way to screening and prevention.
Although modern medicine can’t yet keep vasculitis from happening, it often can keep vasculitis from getting worse. Early diagnosis and appropriate treatment will give your child the best possible chance of avoiding potential complications of this disease, as well as sending it into remission as soon as possible.
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.
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.
Q: Why did my child get vasculitis?
A: We don’t know exactly why some children’s immune systems turn against their own bodies -- specifically their blood vessels, in the case of vasculitis -- but researchers believe it’s caused by a combination of genetic and environmental factors. It’s important to remember that your child’s vasculitis wasn’t caused by anything you did, and there’s nothing you could have done to prevent it.
Q: Are my other children at risk for developing vasculitis? Should they be tested?
A: Despite the fact that genes probably play a role in vasculitis, it’s unusual for this illness to occur in more than one member of the same family. And testing to try to identify vasculitis in anyone isn’t recommended unless there are specific symptoms or medical problems that the test results can be weighed against.
Q: What kinds of doctors treat vasculitis?
A: The doctors who specialize in inflammatory diseases of the muscles, joints and connective tissues are called rheumatologists; they typically diagnose and set up the treatment plan for a child with vasculitis. However, other kinds of specialists may also be involved, including nephrologists (kidneys); pulmonologists (heart); neurologists (central nervous system); dermatologists (skin); and gastroenterologists (digestive tract).
Q: If my child is treated and his symptoms go away, does that mean he’s cured?
A: Vasculitis is considered a lifelong condition: While it can go into remission (that is, the disease is no longer active), it’s not “cured.” The symptoms may one day return (called relapse), or they may never come back -- much depends on the type of vasculitis your child has.
Q: Will my child need to be on a special diet?
A: There isn’t any evidence that eating specific foods or taking special supplements will actually change the course of this illness (that is, make it better or worse). But you can support your child’s overall good health by making sure he eats a balanced, “heart-healthy” diet: plenty of fruits and vegetables, whole grains, low-fat dairy products and lean sources of protein. And load up on Vitamin D and calcium, especially if your child’s treatment includes corticosteroids, which can weaken bones.
Q: Will vasculitis affect how active my child is?
A:Broadly speaking, vasculitis can cause kids to tire more easily, and it’s important for them to rest when they need to. But you should always encourage your child to get regular exercise to help keep his body strong.
When to seek medical advice
The symptoms that often appear in early stages of vasculitis -- fever, fatigue, weight loss, muscle aches and so -- are by no means proof that your child has this disease. However, they do mean something is making him ill and needs medical attention. You should take your child to see his pediatrician, who will refer you to a pediatric rheumatologist if vasculitis is suspected.
Questions to ask your doctor
You and your family are key players in your child’s medical care. It’s important that you share your observations and ideas with your child’s health care provider, and that you understand your provider’s recommendations.
If you’ve made an appointment to talk to a rheumatologist about your child’s vasculitis, you probably already have some ideas and questions on your mind. But at the appointment, it can be easy to forget the questions you wanted to ask. It’s often helpful to jot them down ahead of time so that you can leave the appointment feeling that you have the information you need.
If your child is old enough, you may want to suggest that he write down what he wants to ask his health care provider, too.
Some of the questions you may want to ask include:
- What kind of vasculitis does my child have?
- How much experience do you have treating this illness?
- What are the possible complications?
- What are the treatment options?
- How will we know whether the treatment is working?
- What are the names of the medications -- can you write them down for me?
- What are their side effects?
- How will this illness affect my child’s day-to-day life?
- What is the long-term outlook for my child?
- What can I do to help support my child’s health and help him cope with his condition?
Useful medical terms
Angiitis, arteritis: Synonyms for vasculitis.
Aneurysm: An abnormal swelling or bulge in a weakened blood vessel wall; if it tears (ruptures), it causes dangerous bleeding inside the body.
Angiogram: A special kind of x-ray used to examine blood vessels.
Artery: A type of blood vessel that carries blood from the heart to the rest of the body; the main artery is the aorta.
Capillary: A type of very small blood vessel that connects arteries with veins.
Flare: When the signs and symptoms of a disease suddenly reappear or worsen.
Infarct: An area of dead tissue caused by lack of blood flow.
Infiltrates: A collection of inflammatory cells within a body tissue; for example, pulmonary infiltrates are visible on chest x-rays in some forms of vasculitis.
Inflammation:A normal reaction by the body to injury or disease, which results in swelling, redness, swelling and sometimes soreness. Vasculitis is inflammation within the blood vessels.
Ischemia: Decreased blood flow to a part of the body.
Occlusion: A blockage within a blood vessel.
Purpura: Purplish spots or patches on the skin or mucous membranes caused by the hemorrhage of small blood vessels in the skin.
Remission: When the signs and symptoms of a disease disappear.
Stenosis: A constriction or narrowing of a blood vessel.
Systemic: Affecting the whole body.
Thrombosis: A blood clot.
Vasculitides: A term used to refer to vasculitis in the plural (that is, instead of saying “one of the types of vasculitis,” a doctor may say “one of the vasculitides”).
Vein: A blood vessel that carries blood back to the heart.