Lupus in Children
Child-onset lupus is quite rare, but can be more serious than adult-onset form of the disease. This article describes the latest information for those who live with lupus at a very early age.
Lupus tends to be considered an adult or young adult disease, yet it is estimated that over 10% of those with SLE are less than 18 years of age. The actual numbers are difficult to determine, but it is calculated that between 5,000 and 10,000 children in the United States have SLE. The actual numbers are difficult to determine, especially on the global perspective, but the Arthritis Foundation estimates that up to 10,000 children and teens in the United States have lupus.
Researchers and healthcare professionals have several names for this form of lupus: childhood lupus (cSLE), juvenile lupus (jSLE) or pediatric lupus (pSLE). It occurs more often in adolescent girls than adolescent boys, but in pre-pubescent children, girls are affected only slightly more than boys of similar age. Like adults who have lupus, children who have lupus are predominantly of African-American, Hispanic, Southeast Asian and North American First Nations descent.
Although many aspects and symptoms of lupus are the same for both children and adults, there are some significant differences. Child-onset lupus can be more severe, and, of course, if someone begins living with this condition earlier in life, there is a greater potential for developing organ damage. For children with lupus and their caregivers, the more that we know about this particular form of this disease, the better we (and they) can advocate for the best care and treatments.
Symptoms and Causes
Boston Children’s Hospital refers to lupus in children as a “shifty disease” because symptoms can change and can be unpredictable. Symptoms are similar to those of adults and may include:
- Loss of appetite
- Weight loss
- Achy and swollen joints and/or muscles
- High fever
- Malar or “butterfly” rash
- Mouth and/or nose ulcers
- Symptoms of Raynaud’s Phenomenon
A child’s organ systems, however, may be more susceptible to involvement and damage than an adult’s. These more severe symptoms may include:
Cagri Toruner, M.D. of Nationwide Children’s Hospital treats children with chronic autoimmune disease and points out that “kidney involvement is present in most children with SLE and is a major complication of the long-term outcome of this disease.” According to a 2016 study published in the Central European Journal of Immunology,” approximately 37-82% of children with SLE will develop lupus nephritis. Your child’s healthcare practitioner may perform a renal biopsy to determine whether or not nephritis is present and to what extent in order to determine the best course of treatment.
It is imperative to always seek medical attention immediately if your child’s health suddenly and drastically changes.
A child can’t “catch” lupus – it is not contagious. There may also be no clear rhyme or reason as to why a child develops lupus. Boston Children’s Hospital makes the interesting note that even in the case of twins, for example, one twin may develop lupus while the other won’t. Many factors may be involved in why a child develops this chronic illness.
It is unclear, however, as to what one thing may cause a child to develop lupus. The Cleveland Clinic suggests that a child may develop lupus due to a number of factors – it may be a combination of environmental and genetic factors. A child may be genetically predisposed to lupus, but that doesn’t mean he or she will necessarily develop it. Something external may trigger its development or even other physiological changes in tandem with genetic predisposition including:
Hormonal fluctuations during puberty may play a key role in the development of lupus in children, especially in young girls. According to the Johns Hopkins Lupus Center, women are “9 times more likely than men to develop lupus.” The female body relies on estrogen to function properly and in fact, estrogen is considered “immunoenhancing.” Women, therefore, typically have stronger immune systems than men most likely because of women’s ability to bear children. Women and girls, however, are also way more likely to develop lupus than men.
Diagnosing Lupus in Children
There is no one single test to definitively diagnose lupus. Your child’s healthcare practitioner will perform several tests to determine if your child may have lupus. Boston Children’s Hospital recommends the following testing be completed in order to more accurately determine why your child is not feeling well:
- A Complete Blood Count (CBC) to analyze the ratio of white blood cells to red.
- An antinuclear antibody (ANA) test to check for the presence of abnormal proteins.
- An anti-DNA test may indicate the presence of lupus nephritis.
- A complement (C3 and C4) test to check for low levels of complement which may increase the risk for infection and indicate lupus.
- A C-reactive protein (CRP) test that will show if there are low levels of CRP in your child’s blood which may indicate inflammation.
- An erythrocyte sedimentation rate (ESR or sed rate) test that will indicate how rapidly red blood cells may fall to the bottom of a test tube – an indicator of inflammation in the body.
Your child’s healthcare practitioner may also test your child’s urine in order to look at kidney function and check for damage, and they may order x-rays to look at your child’s internal organs, tissues and bones.
The American College of Rheumatology no longer uses specific criteria for the diagnosis of lupus, however these 11 criteria are used by healthcare practitioners as a guide for determining the kinds of treatments that may be needed. The they include:
- Malar or “butterfly” rash.
- Discoid rash.
- Mouth ulcers.
- Joint inflammation.
- Heart and lung complications.
- Kidney issues.
- Neurological problems including seizures.
- Positive blood tests.
- Changes in normal blood values.
To date, there is no specific mention as to whether or not the existing criteria mentioned above will change for diagnosing lupus in children as well. We will publish updates as information becomes available.
What is the treatment for lupus in children?
Treating pediatric lupus is a team-approach. The Children’s Hospital of Philadelphia suggests that your child’s pediatric healthcare team may change depending on the health of your child and the symptoms or conditions they are experiencing. A 2019 study published in Pediatric Rheumatology stressed that “there is a strong need for multidisciplinary collaboration” when it comes to researching and treating pediatric lupus. Researchers in the 2019 study also concluded that there is a huge need for more collaboration specifically with nephrologists and dermatologists and that the need for mental health specialists should not be overlooked. This study “represents a first research prioritization exercise effort among rheumatologists, dermatologists and nephrologists aimed at identifying key research opportunities and improving outcomes for cSLE.”
Your child will most definitely need their pediatrician and a rheumatologist on their team. Other team members may include:
- A nephrologist if lupus affects kidney function.
- A dermatologist to treat any rashes, dermatitis or other skin conditions.
- A cardiologist if your child develops any kind of heart or cardiovascular conditions.
- A neurologist if you child experiences seizures or any other conditions that affect the brain.
Treating lupus in a child is similar to treating it in an adult. Boston Children’s Hospital lists the following treatment options:
- Corticosteroids (prednisone) to suppress the entire immune system.
- Steroid-sparing therapies (methotrexate, azathioprine) that work similarly to corticosteroids but without all the same side effects.
- Biologics (rituximab, belimumab) that only target certain parts of the immune system.
Note: As of 2019, Benlysta (belimumab) has been approved for children as young as 5 years old in Japan, Europe and the United States.
- Cytotoxins (cyclophosphamide) that destroy the dividing cells in an overactive immune system.
- Intravenous immunoglobulin (IVIg) that is made up of healthy antibodies to help get the immune system back on track.
Boston Children’s Hospital also suggests that alternative therapies such as acupuncture and meditation may help, but to always discuss these options first with your child’s healthcare team.
Helping your child adopt a healthy lifestyle will help stave off symptoms and flares. A healthy lifestyle includes:
- A healthy diet and maintaining a healthy weight.
- Staying active as much as possible.
- Limiting sun exposure and using adequate protection against UV exposure.
- Getting plenty of rest and sleep.
What is the prognosis or long-term outlook for children diagnosed with lupus?
A 2012 study published in the journal Pediatric Clinics of North America states that pediatric lupus outcomes have improved significantly over the years with a 10 to 15-year survival rate exceeding 85%.
It is important to note, however, that with longer survival rates comes an increased risk of prematurely developing atherosclerosis, myocardial infarction, and/or osteoporosis. Your child may also be at a greater risk for developing end-stage renal disease (ESRD) or even lymphoma due to an increase in the risk of malignancy. Researchers of the study also found that about one-third of children with pediatric lupus reported that their health negatively impacted their education which could in turn impact the ability to work as an adult. All of this makes it even more important to learn as much as you can and advocate for your child with your child’s healthcare team to ensure your child leads the healthiest life possible and receives the best, treatment targeted towards their specific and unique needs.
The tools and indexes pediatric healthcare practitioners use to evaluate how well a child is doing and responding to treatment, however, need to be updated. A 2019 American College of Rheumatology study published in the journal Arthritis Care & Research acknowledges this fact and developed the Childhood Lupus Improvement Index (CHILI) as a “tool to measure response to therapy in childhood-onset systematic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRI).” Researchers claim this new and improved indexing tool is a “seemingly accurate” way to evaluate a child’s health in response to cSLE therapy.
Helping your child cope with a lupus diagnosis
Living with chronic illness can take its toll on the entire family. It’s important to get as much information as you can about your child’s illness in order to explain what’s going on to other family members, but first and foremost, your child. You want to be your child’s best advocate.
A pediatric lupus diagnosis can be stressful and emotional – this is perfectly normal and to be expected. The American Psychological Association (APA) also notes that parents may experience a gamut of emotions from anger and sadness to guilt. The APA suggests making sure you work to gain control over your emotions and develop strong stress management tools – it may be advantageous for you to seek the help of a mental health therapist for both you and your child. If you can’t control your emotions, it’s likely your child won’t be able to control theirs, either. Your entire family may even benefit from mental health counseling, especially at the very beginning.
Amanda Thompson, psychologist and Director of Patient Support Services at the Center for Cancer and Blood Disorders, suggests the following five tips to follow when talking with your child about chronic illness:
- Having the conversation sooner rather than later – Children are wise and can tell when something is wrong or you are holding something back. Knowledge will help take away some of the mystery and fear.
- Call the illness by its name – Thompson opposes “sugarcoating” chronic illness. While neither you nor your child may fully understand what lupus means or looks like, make sure you both start to become familiar with it right away.
- Keep talking with your child – Telling your child everything all at once will probably be too much, especially if your child is very young. Tell them a little at a time, but keep telling them and leave yourself open to questions even if you don’t have all the answers. Your child will begin to process the information over time in a way that’s easy for them to cope.
- Provide reassurance – Make sure you let your child knows that you will be there for them no matter what, and that developing lupus is not their fault. Sometimes children think getting sick or hurt is “punishment” for misbehaving. Assure them it’s not. You want your child to feel as secure and as safe as possible.
- Be specific about the impact the illness will have on your child and family – Be upfront with your child so they know what they may be facing and what to expect. There will undoubtedly be missed school days, extra healthcare visits and new medications. Your child may not be able to participate in all the activities they once did, and this can be upsetting. If your child is prepared for lifestyle changes – while it still may be stressPful – it shouldn’t come as a complete surprise.
The University of Michigan reminds parents that the siblings of a sick child often feel neglected. Siblings may even feel angry or resentful of their parents or their brother or sister that seems to be getting all of the attention. It is important to remind all of your children how important and special they are – never assume they just “know.” Take the time to speak the words and show the love!
Lupus in a child can be just as mysterious and unpredictable as it can in an adult. It’s vital to remember that knowledge can eliminate a lot of fear. Sure, you and your child may be scared at times and this is to be expected, but facing your fears together – and as a family – will go a long way in bravely battling this chronic disease.
Ardoin, S., Daly, R., Merzoug, L., Tse, K., Ardalan, K., Arkin, L., …Hersh, A. (2019). Research priorities in childhood-onset lupus: results of a multidisciplinary prioritization exercise. Pediatric Rheumatology 17(32) Retrieved from: https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-019-0327-4
Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., … Johnson, S., (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis & Rheumatology. Retrieved from: https://ard.bmj.com/content/annrheumdis/78/9/1151.full.pdf
Brunner, H., Holland, M., Beresford, M., Ardoin, S., Appenzeller, S., Silva, C., … Ying, J. (2019). American College of Rheumatology provisional criteria for clinically relevant improvement in children and adolescents with childhood-onset systemic lupus erythematosus. Arthritis Care & Research. Retrieved from: https://www.rheumatology.org/Portals/0/Files/Provisional-Criteria-Clinically-Relevant-Improvement-Childhood-Onset-SLE.pdf
Causes of lupus. (2019). Retrieved from: https://www.hopkinslupus.org/lupus-info/lupus/
Children with chronic conditions. (2019). Retrieved from: http://www.med.umich.edu/yourchild/topics/chronic.htm
Levy, D., & Kamphuis, S. (2012). Systemic lupus erythematosus in children and adolescents. Pediatric Clinics of North America. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3348509/pdf/nihms-362971.pdf
Lupus. (2019). Retrieved from: https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Lupus
Pediatric lupus. (2019). Retrieved from: https://www.chop.edu/conditions-diseases/systemic-lupus-erythematosus-lupus
Pineles, D., Valente, A., Warren, B., Peterson, M.G.E., Lehman, T.J.A., & Moorthy, L.N. (2011). Worldwide incidence and prevalence of pediatric onset systemic lupus erythematosus. Lupus, 20, 1187-1182.
Silverman, E., (2007). Pediatric systemic lupus erythmatosus. Future Rheumatology, 2(1) DOI: http://dx.doi.org.proxy.lib.pdx.edu/10.2217/174608126.96.36.199
Systemic lupus erythematosus (lupus) in children and teens. (n.d.). Retrieved from: https://www.arthritis.org/about-arthritis/types/lupus-in-children-and-teens/
Systemic lupus erythematosus (SLE) in children. (2019). Retrieved from: https://my.clevelandclinic.org/health/diseases/14796-systemic-lupus-erythematosus-sle-in-children
Szymanik-Grezelak, H., Kuzma-Mroczkowska, K., Maldyk, J., & Panczyk-Tomaszewska, M. (2016). Lupus nephritis in children – 10 years’ experience. Central European Journal of Immunology. Retrieved from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5099380/
Thompson, A. (2017). 5 tips for talking to your child about disease. Retrieved from: https://childrensnational.org/news-and-events/our-blogs/parenting-blog/2017/september/5-tips-for-talking-to-your-child-about-disease
Toruner, C. (2017). Lupus and kids: Understanding child onset disease. Retrieved from: https://www.nationwidechildrens.org/family-resources-education/700childrens/2017/09/lupus-and-kids-understanding-child-onset-disease
What are the symptoms of lupus? (n.d.). Retrieved from: http://www.childrenshospital.org/conditions-and-treatments/conditions/l/lupus/symptoms-and-causes
When your child is diagnosed with chronic illness. (2019). Retrieved from: https://www.apa.org/helpcenter/chronic-illness-child
Author: Liz Heintz
Liz Heintz is a technical and creative writer who received her BA in Communications, Advocacy, and Relational Communications from Marylhurst University in Lake Oswego, Oregon. She most recently worked for several years in the healthcare industry. A native of San Francisco, California, Liz now calls the beautiful Pacific Northwest home.
All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at [email protected] for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus.
All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.