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Vasculitis

  • Overview

    Robert Sundel, MD

    Day in and day out, tens of thousands of miles of blood vessels carry oxygen and nutrients to every part of your child’s body. When blood vessels become inflamed -- a condition called vasculitis -- it can slow or even shut down these vital deliveries. Vasculitis is generally rare in children, but when it occurs doctors take it very seriously, because of its potential for damaging still-growing organs and tissues.

    • “Vasculitis” is a general term for inflammation of the blood vessels, which include arteries, veins and capillaries.
    • With inflammation -- a process that causes swelling and irritation -- blood vessels may become narrow or even blocked. Inflammation can also weaken blood vessel walls.
    • There are many forms of vasculitis, ranging from mild to potentially life-threatening, because the inflammation may:
    • affect blood vessels of any type or size
    • involve a single organ or many organs and tissues, including the heart, brain and kidneys
    • The most common form of vasculitis in children is Henoch-Schönlein purpura (HSP), which affects blood vessels in the skin, intestines and often the kidneys.
    • The second most common form in children is Kawasaki disease, which affects blood vessels in many parts of the body, often those around the heart.
    • HSP, Kawasaki disease and other forms of vasculitis are autoimmune illnesses, meaning that -- for reasons doctors don’t yet fully understand -- the immune system is mistakenly attacking the body itself.
       

    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.
     

    How Children’s Hospital Boston approaches vasculitis

    Children’s is famed as a national referral center for Kawasaki disease, and most of the current therapies for this illnesses were either initiated or validated here. But we successfully treat many other kinds of childhood vasculitis as well, building on these strengths:

    • 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 Disease 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, whichunites 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 whom a single diagnosis doesn’t fit.

    Reviewed by Robert Sundel, MD
    © Children’s Hospital Boston, 2011


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    Boston Children's Hospital 
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    Boston MA 02115
     617-355-6117
  • In-Depth

    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 purpuraoccurs 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.
     

    Causes

    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.
     

    Symptoms

    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
       

    Prevention

    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.
     

    Complications

    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.
     

    Long-term outlook

    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.
     

    FAQs

    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.

    Hemorrhage: Bleeding.

    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.

  • Tests

    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 teststo 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 teststo check for the presence of red blood cells or high levels of proteins, which may indicate that vasculitis is affecting the kidneys
    • imaging teststo 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.

  • It's understandable that you may feel upset or frightened to hear your child has vasculitis. But at Children's, we view the diagnosis as a starting point: Having identified your child's condition, we're able to begin the process of treating him so that we may ultimately return him to good 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 immunosuppressantsoffer 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).
    • Biologicsare 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.
     

    Coping and support

    We understand that you may have a lot of questions when your child is diagnosed with vasculitis. How will it affect my child's life? What do we do next? We've tried to provide some answers to those questions here, but there are also a number of other resources to help you and your family in dealing with your child's unique illness, such as:

    • Parent-to-parent connections: Want to talk with someone whose son or daughter has faced the same kind of vasculitis as your child? A number of Children's parents volunteer for special training to help the families of newly diagnosed kids. Alternatively, your child's doctor may be able to put you in touch with a mom or dad of another patient who can share their experiences with you.
    • Social work: Our Rheumatology Department includes social workers -- in both inpatient and outpatient settings -- who have assisted many other families whose children have vasculitis. Your social worker can offer counseling and problem-solving advice on issues such as coping with your child's diagnosis; dealing with financial difficulties; and finding temporary housing near the hospital if your family is traveling to Boston from another area.
    • Coping Clinic: This program has an experienced team of pediatric psychologists, psychiatrists and other mental health professionals to help children and families deal with any extra stress that vasculitis may bring. Offering evaluations, short-term therapy and family counseling, the Coping Clinic staff can teach you ways to prevent or better deal with the challenges of long-term illness.
       

    In addition, there are many groups that help connect and educate people across the country who have vasculitis. Your doctor may be able to recommend which ones are best for your child's specific kind of vasculitis, but a good place to start is the Vasculitis Foundation (www.vasculitisfoundation.org), an international nonprofit with over 4,000 members in 58 countries. The group provides educational materials on all kinds of vasculitis, as well as contact information for local support groups. The foundation also encourages and supports research into the cause, cure and treatment of vasculitis.

  • Research & Innovation

    As a national referral center for Kawasaki disease, Children’s Hospital Boston is on the forefront of treatments for this type of vasculitis. Most of the current therapies for Kawasaki, in fact, were either initiated or validated here. But advances in diagnosis are equally important: Without timely treatment, Kawasaki may inflame the coronary arteries and lead to potentially life-threatening heart problems. Children’s researchersare now working to identify proteins in urine that may act as a biomarker -- or red flag -- for whether a child has Kawasaki, and allow treatment to begin as soon as possible.

    Through clinical trials and research projects, Children’s is also helping to pioneer the use of biologics -- protein-based drugs made from living cells -- for a number of pediatric diseases, including vasculitis. Most recently, our rheumatologists examined a class of biologics called tumor necrosis factor (TNF) blockers as a treatment for Takayasu arteritis. In a study of Children’s patients, they found that TNF blockers worked reliably with other medications to help sustain remission in Takayasu patients.

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