What is lupus?

Like all autoimmune diseases, lupus causes the immune system — our natural protection against foreign invaders like viruses and bacteria — to mistakenly attack the body itself. What makes lupus unusual, and frequently distressing for patients and families, is its unpredictability: It can affect almost any part of the body, and often many parts at the same time. There are different types of lupus, but in general the word “lupus” is shorthand for the most dominant form, systemic lupus erythematosus. Systemic means it can involve many parts of the body. Erythematosus comes from the Greek word for “red,” and describes the lupus characteristic cheek rash. Lupus is Latin for “wolf,” which some believe refers to the way a check rash resembles a wolf bite.

Also known as SLE, this illness:

  • is a chronic (meaning lifelong) autoimmune disorder with no known cause or cure
  • affects about 5 million people worldwide, most often adolescent girls and younger women (15 to 44)
  • can target various parts of the body, including the skin, joints, blood, and vital organs like the kidneys, heart, lungs, and brain
  • tends to alternate between being more active (when symptoms surge, or flare) and less active (when symptoms appear to go away)
  • is less common in children

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. Back in the 1950s, children with lupus faced only about a 30 to 40 percent chance of survival. Today, however, there are powerful medications that can bring this illness under control — often permanently — in the majority of children and allow them to lead full, relatively normal lives.

Systemic lupus erythematosus

When SLE occurs in children doctors tend to call it pediatric systemic lupus erythematosus (pSLE) because it typically hits kids harder than adults and carries extra health risks, since children have more years to accrue organ damage compared with adults. About 15 percent of SLE patients are younger than 18. In the U.S., the disease affects an estimated 5,000 to 10,000 youngsters. Children with lupus:

  • 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)

What are the other forms of lupus?

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, his or her doctor will talk with you in detail about what this diagnosis means, and what the next steps will be.

What are the symptoms of lupus?

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:

  • fatigue
  • 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) and 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)

What causes 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:

  • Family history: A child is born with certain genes that make him or 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.

How we care for lupus

If your child has lupus, it can be a confusing and scary diagnosis to deal with. At Boston Children’s Rheumatology Program, we will be with you every step of the way in fighting this disease and helping your child enter adulthood as healthy as possible. As a partner in this process, we offer experience, specialists who have dealt extensively with childhood lupus, and research and innovation that lends itself to the treatment and management of lupus in children.