When people use the term “lupus,” they’re usually referring to systemic lupus erythematosus (SLE), which affects about 5 million people worldwide. Systemic means it can involve many parts of the body. Erythematosus comes from the Greek word for “red,” and describes lupus’ characteristic malar (cheek) rash. Lupus is Latin for “wolf,” which some believe refers to the way a malar rash resembles a wolf bite.
- SLE affects each person differently, and can range in severity from mild (aches and rashes) to life-threatening (damage to vital organs). But the underlying process is the same in everyone:
- The immune system becomes confused: It loses its ability to tell the difference between a foreign invader and the body’s own cells and tissues.
- It begins sending out autoantibodies—special blood proteins that mark invaders for attack—that actually target specific healthy cells and tissues in the body. Because these antibodies are working against one’s own self, they’re called autoantibodies.
- The autoantibodies trigger other parts of the immune system to join in, causing inflammation—the redness, warmth and swelling that happens as blood vessels widen to pour fighter cells into the attack site.
- The immune system then fails to call off the mistaken attack. Until it does so, the inflammation continues “burning,” which can damage or even destroy the targeted tissues and cells.
With lupus, doctors can’t predict what part of the body the immune system will choose to strike, or when. But they can use medications to help prevent or blunt these attacks, and to extinguish the harmful inflammation.
SLE in children (pSLE)
About 15 percent of SLE patients are younger than 18; in the United States, the disease affects an estimated 5,000 to 10,000 youngsters. Doctors call this pediatric systemic lupus erythematosus (sPSLE) to underscore the different challenges these kids face when compared with adults. Namely, children with lupus:
- generally have more severe symptoms when they are diagnosed
- are more likely to have problems with vital organ systems—most critically, the kidneys and the central nervous system (brain and spinal cord)
- develop damage from their disease more quickly
- have a higher “burden of disease” over their lifetime (meaning that the earlier their lupus begins, the more years they spend living with it)
The outlook for children with lupus can vary a great deal, however, depending on when the disease begins (called the onset).
- age 15-18 (more common): In this group, pSLE tends to look and behave more like adult lupus; its severity can range from mild symptoms to life-threatening disease.
- younger than 15 ( less common): Symptoms can be more severe in this group, and there’s a greater chance that vital organs will be affected.
- younger than than 5 (rare):The smallest children are typically among the sickest lupus patients, largely because most also have complement deficiency, meaning they don’t have enough of certain blood proteins that play a key role in the immune system.
It’s important to remember that everyone with lupus responds to the disease—and the medications used to treat it—in his own unique way. You can’t predict exactly how lupus will progress in your child. But you can greatly improve your child’s odds for a healthy future by helping her understand and stick with the recommended treatments for her condition.
Other forms of lupus
Although this article deals mainly with systemic lupus erythematosus, Children’s Hospital Boston does treat youngsters with less common forms of lupus. These include:
- Drug-induced lupus (DIL): This lupus-like illness can crop up in people who take certain medications for a long time. DIL is fairly rare in children, and when it occurs the most common culprit is the acne drug minocycline. DIL has some of the same symptoms of lupus—fever, fatigue, joint and muscle stiffness—but doesn’t tend to affect vital organs. Symptoms usually disappear within a few weeks after the “triggering” medication is stopped.
- Neonatal lupus: This temporary form of lupus affects a small percentage of infants whose mothers have certain lupus autoantibodies. It begins before birth, when these autoantibodies reach the baby via the placenta, and ends within the first several months of life, as the autoantibodies disappear from the baby’s system. The symptoms—skin rash, low blood cell counts—are likewise temporary. However, neonatal lupus does have the potential to cause permanent damage to the baby’s heart (called heart block). If the heart block is significant, a baby may need a pacemaker.
If your child has —or may have—drug-induced lupus or neonatal lupus, her doctor will talk with you in detail about what this diagnosis means, and what the next steps will be.
Who is at risk?
Researchers are still working to unlock the mysteries of why and how lupus happens—and who is likely to develop it. However, when we look at people who already have this disease, four risk factors emerge:
- Age:Though lupus can affect all ages, including infants and children, it most often occurs between ages 15 and 44.
- Gender:Women make up more than 90 percent of adults (18 to 44) who are diagnosed with lupus. There is much less of a gender difference when lupus occurs before adolescence.
- Race:Lupus is two to three times more common among African Americans, Hispanics, Asians and American Indians. It also appears to occur at an earlier age and be more severe in these groups.
- Family history:Heredity does seem to play a role, though not an overly strong one—only about 10 percent of people with lupus also have a close relative with lupus.
We don’t yet know why some children develop lupus and others don’t. It’s not contagious, like measles—you can’t “catch” it from another person. It’s not a disease that parents pass directly down to their children; in fact, there’s only about a 5 percent chance that a son or daughter of someone with lupus will also develop it.
While researchers do believe that genes play a big role in causing lupus, there’s more to it than that. Otherwise, you’d expect that if one identical twin has lupus, the other would, too— but that’s often not the case. Instead, there’s likely a two-part process involved in causing lupus:
- Heredity: A child is born with certain genes that make her susceptible to lupus. Think of a forest in dry, hot weather: The ingredients for a wildfire are there, but it takes something else to spark the blaze.
- Environmental factors: The child encounters something—or a combination of things—that causes the disease to “ignite.” The environmental factors that may trigger lupus include infections, ultraviolet light and perhaps extreme stress. And given that so many lupus patients are female, it’s also likely that hormones play an important role in the development of and risk for this disease. However, there’s still a lot we don’t know about these triggers, especially why some affect certain children and not others.
Scientists are now working to discover which genes are involved in lupus—and how its potential disease triggers work—in order to bring us closer to curing or even preventing this chronic illness.
Signs and symptoms
Lupus is known as “the great imitator” because many of its earliest warning signs are common in other illnesses, too. Fever, low energy, no appetite? It could be the beginning of lupus—or it might just be the flu.
Lupus is also a very shifty disease: Symptoms often come and go; new ones may crop up, while others seem to disappear. Symptoms also vary greatly from person to person, depending on what part of the body the disease is affecting at the time.
For all these reasons, diagnosing childhood lupus often requires the expertise of pediatric rheumatologists. These specialists are the best qualified to sort out the signs and symptoms of lupus from other diseases, so your child’s treatment can begin as quickly as possible.
Common symptoms of lupus include:
- loss of appetite
- weight loss
- swollen or achy joints
- muscle aches
- fever of over 100 degrees F
- skin rashes, especially:
- a butterfly-shaped rash across the cheeks (this so-called malar rash is a hallmark of lupus)
- rashes that develop on sun-exposed skin
- brittle hair, or unusual hair loss
- ulcers in the mouth or nose
- fingers that turn white and/or blue from cold or stress (Raynaud’s phenomenon)
Compared with adults, children with lupus are more likely to have problems with vital organs, especially the kidneys and the brain. These symptoms may include:
- dark urine; swelling around the feet, legs and eyelids (kidney inflammation, or nephritis)
- shortness of breath, chest pain (lung inflammation, or pleuritis)
- headaches, memory problems, seizures (brain inflammation, or cerebritis)
Being diagnosed with lupus as a teenager is especially tough. You want to fit in at school, but suddenly you have a illness that might make you feel very different from everyone else. Probably no one else in your class has it, or even knows much about it—how it can make you tired even when you look okay, or how the medications you take can make your face break out or look puffy.
While having a chronic illness may leaving you feeling isolated, it’s important to remember that lupus doesn’t mean you can’t hang out with your friends, go on dates or play sports. And hey, you’re still you—the people who liked you before you got sick will still like you now, and they’ll want to help you feel better any way they can. Plus, you’ll have a lot of fresh backup: Doctors, counselors, physical therapists and other members of your treatment team are all there to support you.
There’s no doubt that having lupus can be an emotional roller coaster. But you can smooth out the ride by keeping a few tips in mind:
Don’t let lupus get the upper hand.On the days when you’re feeling pretty good, you may be tempted to skip your medications or doctor’s appointment. Don’t be fooled! Your lupus is still there, only quiet—and if you want to keep it quiet, you need to stick with your treatment plan day in and day out, along with eating right and getting enough exercise and rest. By doing these things, you may end up feeling more in control of your disease, and not the other way around.
Whoa…pace yourself! With lupus, you may have a limited amount of energy to spend. Try to take things at a steady pace so you get done what you need and want to do. Whether it’s studying, exercising or even shopping, stop before you drop. Ask for help when you need it. Listen to your body and, if you feel tired, take a break.
If it sounds like a bad idea, it probably is. Smoking, drinking and taking drugs can seriously mess up your medications and may make your symptoms flare up. Tattooing and body piercing are also off limits because of an increased risk of infection. But if you’re dying to rebel against your parents, take heart: There’s a lot of music out there that adults can’t stand, and nothing’s stopping you from cranking that up.
Check in with your doctor.It’s vital that your rheumatologist be not just an expert on lupus, but also an expert on you—from figuring out which medications work best for you to spotting any new symptoms that pop up. You are your doctor’s number-one source of information, so don’t be shy about sharing what’s going on with you. For instance, if you’re beginning to think about going on “the Pill,” talk it over with your rheumatologist first, because oral contraceptives pose a higher risk of blood clots for some lupus patients.
Let your feelings show. There’s no getting around the fact that some days you’re going to feel angry or depressed about having lupus. That’s normal, and being able to talk it out with your friends or family can help you through the rough patches. But—especially if these feelings don’t go away— you may also want to talk with a counselor or therapist. Staying healthy in body and mind is the best way to beat your disease.
Talk to someone who’s been there. Connecting with other teens with lupus is a great way to not only make new friends, but also to pick up tips and strategies for dealing with your illness. Ask your doctor if you might be able to meet up with another patient your age, or check out the social networking site Starbright World, which offers teens with all kinds of chronic illnesses a way to link up and hang out together online.
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 lupus, 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 she write down what she wants to ask her health care provider, too.
Some of the questions you may want to ask include:
- How much experience do you have treating pediatric lupus?
- What part of my child’s body is the lupus affecting right now?
- 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 her cope with her condition?
Q: Why did my child get lupus?
A: We don’t know exactly why some children’s immune systems begin attacking their own bodies. It’s related to something in their genes and likely a number of other, unknown factors. It’s important to remember that your child’s lupus wasn’t caused by anything you did, and there’s nothing you could have done to prevent it.
Q: Will my other children get lupus, too? Can they be tested?
A: Though lupus isn’t a disease that passes directly from parent to child, it does tend to run in families. However, the risk is low enough that it’s very rare for two siblings to have lupus. And while there are blood tests that can help doctors make a diagnosis of lupus, they don’t provide useful information about children who have shown no symptoms of disease.
Q: Can vaccinations trigger lupus?
A:Vaccinations DO NOT cause this disease. Moreover, if your child has lupus, it’s important that she continue receiving vaccinations against things like tetanus, flu and pneumococcal disease, because both her illness and the medications used to treat it can leave her more vulnerable to infections. The only exceptions to this rule are “live” vaccines—like MMR (measles, mumps and rubella) and nasal flu sprays—which can be harmful for kids with a suppressed immune system.
Q: Is there a link between lupus and aspartame?
A: There is a lot of misinformation about lupus on the Web, including a widely circulated warning that the artificial sweetener aspartame causes lupus. But there is absolutely no scientific proof that aspartame , or anything in our diet, for that matter, causes lupus or makes it worse.
Q: Is lupus similar to AIDS?
A: Although both diseases involve problems with the immune system, lupus and HIV/AIDS are almost polar opposites: In lupus, the immune system is overactive, while in HIV or AIDS, the immune system is underactive. In addition, lupus is not infectious—unlike AIDS, it can’t spread through sexual contact, blood transfusions or infected needles.
Q: How can lupus be fatal?
A: Lupus is most dangerous when it affects vital organs. In young children, doctors watch closely for any involvement of the kidneys and the central nervous system (the brain and spinal cord), because these two organ systems require the most immunosuppression and, if damaged, can have life-threatening complications.
Over the long run, the leading cause of death among lupus patients is cardiovascular disease. But if children with lupus develop heart-healthy habits early on, they can significantly reduce their risk for this illness later in life.
Q: Will my child need to be on medication her whole life?
A: Kids with very mild cases of lupus may require certain medications only when their symptoms bother them: ibuprofen for achy joints, for instance, or cortisone cream for skin rash. But all lupus patients should take hydroxychloroquine, an antimalarial drug that’s been shown to decrease lupus flares, every day and possibly for the rest of their life.
The good news is that the lupus drugs with the most serious side effects—like corticosteroids—are rarely used over the long term. Instead, doctors aim to get kids off these powerful medications as soon as possible.
Q: How can I find out about clinical trials for lupus?
A: Start by asking your child’s doctor, who will be the best source of information about any new treatment that might benefit your child. You can also learn more about clinical trials at the Lupus Foundation of America’s website to search for current and upcoming trials at Children’s.
Q: Can lupus be prevented?
A:There is no way to keep a child from getting lupus; there are no vaccines against this illness, no special diet to ward it off. But once the disease begins, there many important ways you and your child can help in treating it.
- Stick to the medications. Some drugs used to fight lupus must be taken for a long time, even years. Others, like corticosteroids, may have unpleasant side effects. It’s not unusual for kids to want to skip their medications when they’re feeling better (one study of pharmacy receipts showed that 40 to 50 percent of lupus patients had failed to refill their prescriptions—and those were adults!). But taking all medications as prescribed—what doctors call adherence—is what keeps the brakes on this disease: Skip a dose, and it will pick up speed.
- Alert your doctor to any new symptoms. Firefighters can’t predict where a blaze will happen, but instead swing into action when one breaks out. Battling lupus is a similar process: Doctors can’t predict what parts of the body the disease may choose to affect, so they treat your child based on what’s actually “on fire” (active kidney inflammation, for example). New symptoms may mean lupus has spread to another part of the body, and you’ll want to sound the alarm for your child’s doctors as soon as possible.
- Take steps to avoid flares. The symptoms of lupus tend to alternate between being fairly quiet and then being very active, called a flare. To help keep your child’s symptoms under control, make sure she eats well and gets enough sleep and exercise, and avoids things that can trigger flares, especially undue sun exposure.
Q: What are the potential complications of lupus?
A:For reasons we don’t yet understand, lupus often attacks children’s vital organs, which can cause some of the most serious problems. While it can be distressing to learn about lupus’ potentially life-threatening complications, it may help you understand why doctors often go after this illness with powerful drugs like corticosteroids, which have their own possible risks and side effects.
Potential complications of lupus include:
- kidney disease (called nephritis), which affects about 60 percent of children with lupus. If unchecked, kidney disease can progress to the point where a child needs dialysis or even a transplant. It’s one of the leading causes of death in pediatric lupus patients.
- central nervous system problems (often cerebritis, inflammation of brain tissues), which affect about 20 to 30 percent of children with lupus and can cause mood changes, memory problems, seizure and even stroke.
- hematological (blood) problems, which affect more than half of children with lupus. The most frequent blood-related complication is a lack of red blood cells, or anemia. Other problems include leukopenia, a decreased number of white blood cells, which can lead to infections; and thrombocytopenia, a decreased number of blood platelets, which can cause clotting problems.
- arthritis, or joint inflammation, which affects more than 75 percent of children with lupus. Possible long-term complications of joint inflammation are osteoporosis (weakened bones) and restricted motion of joints.
- respiratory problems (called pleuritis, inflammation of the lining of the lungs), which affect up to half of children with lupus.
- heart problems(often pericarditis, inflammation of the membrane around the heart), which affect up to 25 percent of children with lupus. There is also a higher risk for serious cardiovascular disease later in life.
In addition, all kids with lupus have a greater risk for developing infection. This is because their immune system is not working properly, but also because many lupus medications work by actually suppressing the immune system, further lowering the body’s defenses against invaders like bacteria and viruses.
Q: What is the long-term outlook for my child?
A:Not that long ago, lupus (in both children and adults) was considered an untreatable disease. One study of lupus patients in the 1950s found that less than half were alive four years after diagnosis.
But advances in treatment have made the outlook for today’s lupus patients considerably brighter. While this remains a serious and long-term illness, an estimated 85 percent of people with lupus will live a normal lifespan.
The outlook for your child depends on a great deal on how severe her illness is. Lupus can be mild, causing skin rashes and joint aches, which require minimal treatment. The disease can also be very active, and if it spreads to vital organs it can do a lot of damage—sometimes irreversible. Your doctor will be the best source of information about what form of lupus your child is facing, and what it might take to bring it under control.
Even if your child’s lupus “settles down”—either on its own or through treatment—it never goes away completely. To help keep the disease from flaring up, your child will probably be taking some type of medication for years to come. She’ll also need to see her doctor regularly for checkups. But by helping your child do these things and adopt good health habits for life (avoiding sun exposure, eating well, exercising), you’ll ensure the best possible outlook for her future.