Juvenile Idiopathic Arthritis (JIA): Chronic Management in Children
Medically reviewed by David Ozeri, MDMedically reviewed by David Ozeri, MDJuvenile idiopathic arthritis (JIA) is a joint disease of unknown (idiopathic) cause that affects children. JIA develops when the immune system becomes overactive and causes inflammation. It was previously known as juvenile rheumatoid arthritis. JIA symptoms include chronic joint pain, swelling, and stiffness. Some children may have symptoms over a few months, while others may have symptoms for many years. JIA can sometimes be severe and lead to growth problems, joint damage, and eye inflammation.
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When Juvenile Idiopathic Arthritis BeginsJuvenile idiopathic arthritis has six categories, including:Enthesitis-related arthritis: This type of JIA causes painful inflammation at the entheses—the sites where tendons and ligaments insert into the bone.Oligoarticular: This is the most common form of JIA and affects four or fewer joints.Polyarticular: This is the second most common JIA type, affecting five or more joints. It is divided into two types: rheumatoid factor (RF) negative and RF positive. RF is an antibody(immune protein) found in the bloodwork of people with rheumatoid arthritis (RA).Psoriatic arthritis: This type of JIA affects children with psoriasis, an autoimmune skin condition that causes red or discolored scaly patches.Systemic-onset: This type of JIA is the most severe, affecting about 10% of children with arthritis. It causes joint symptoms and systemic symptoms like fever, lymph node swelling, fatigue, and whole-body inflammation. Undifferentiated arthritis: JIA that falls under two or more types or doesn't match any type is classified as undifferentiated arthritis. Age of DiagnosisJIA affects children 16 and younger. The average age of onset is ages 2 to 5. JIA can also occur in children as young as 6 weeks old. It is possible for a child to outgrow JIA, although it may affect bone development. Some types of JIA affect children of one sex more than another, while others affect children of all sexes equally. Children of all races and backgrounds can have JIA.
Related: Early-Onset Arthritis: Types, Symptoms, and Treatment
Symptom Onset and DurationJIA affects 1 in 1,000 children in the United States. Some children may only experience JIA for a few months, while others may have it for several years. JIA can also become adult arthritis and last a lifetime. It may take years for a child to receive a JIA diagnosis because symptoms may come on slowly. Some early symptoms of JIA are:Appetite lossEye inflammation Fatigue High fevers—103 degrees F and upJoint pain and swellingJoint stiffness in the morning Skin rashSwollen lymph nodesJIA remission occurs when children's symptoms improve or completely disappear. Some children experience remission for months or years, and for others, it could be permanent.But even in permanent remission, a child will need to continue managing JIA into adulthood. Fortunately, JIA treatments and monitoring have improved the prognosis for most children.One study reported in 2014 followed 176 children with JIA for 30 years and found that 59% were in remission without medication at the 30-year mark. Seven percent were in remission with medication, while 34% were still living with an active disease. Joint Symptoms and Long-Term Effects The symptoms a child will experience with JIA will depend on the type of JIA they have. Joint symptoms may include:Changes in function, such as trouble with using writing instruments and utensils and not playing or climbing as the child previously didLimpingMorning joint stiffnessPain, swelling, tenderness, and warmth of jointsReduced range of motion of affected jointsLeft untreated, JIA can have long-term effects. Such effects include:Bone and cartilage damage Difficulty with daily tasks Digestive troubles related to inflammation of the digestive systemGrowth problems related to bone and joint damage Late on-onset puberty Lung or heart problems Spinal inflammation, especially in the cervical spine (neck)Vision problems Risk of JIA in ChildrenJIA is an autoimmune disease, which means the body's immune system malfunctions and attacks its own healthy tissues.The exact causes of JIA are not known, but genes and environmental factors are believed to play a role, as follows:Environmental factors: Exposure to an illness or infection, certain medications, or something in a child's environment can trigger the development of JIA.Genetics: Familial studies have produced estimates on the contribution of genetic factors for JIA, which have ranged from 13% to 25%. JIA is linked to a gene called HLA DR4. JIA and HLA DR4 tend to run in families. Genes alone are not enough to trigger the disease, and environmental risk factors further increase risk. It is also possible for a child to have the HLA DR4 gene and never develop JIA.Sex: Some types of JIA affect more girls and boys, except for enthesitis-related JIA, which affects more boys.
A Note on Gender and Sex TerminologyVerywell Health acknowledges that sex and gender are related concepts, but they are not the same. To accurately reflect our sources, this article uses terms like “female,” “male,” “girl,” and “boy” as the sources use them.
How to Manage FlaresLike adult arthritis, JIA causes periods of high disease activity known as flare-ups or flares. Flare-ups cause severe pain, swollen joints, and additional symptoms like fever, fatigue, skin rash, and eye problems (blurry vision and dry eyes). Ways to help your child manage a flare are:Heat and cold therapies: Warm compresses, heating pads, baths, and showers can help improve blood flow to muscles and joints and reduce stiffness and pain. For inflamed joints, which will be warm to the touch, use an ice pack or a bag of frozen vegetables on the affected joint. Ice can constrict blood vessels to decrease inflammation and pain.Rest and activity: Encourage your child to rest an inflamed and sore joint. Alternating between rest and activity can reduce strain on affected joints and prevent injuries.Use a splint for pain control: Wearing a splint at night might help your child to move an affected joint less. Your child's healthcare provider or a physical therapist can provide information about splints or braces.Reach out to your child's provider: If your child's symptoms are causing them to miss school or other activities, make an appointment with their rheumatologist or other medical provider. A rheumatologist is a medical doctor who specializes in the diagnosis and treatment of diseases affecting the bones, joints, and muscles. Your child's provider can provide additional treatment options for managing the flare or adjust treatments to prevent flares from occurring in the first place. Over-the-counter (OTC) pain relief: Discuss in advance the safety of OTC pain relief for your child with their healthcare provider. OTC pain relief may include Tylenol (acetaminophen), nonsteroidal anti-inflammatory drugs (NSAIDs) like Advil or Motrin (ibuprofen) and Aleve (naproxen sodium), and topical pain relief creams and gels. Pay attention: Children do not always speak up when they are not feeling well and may not have the words to articulate what they are feeling. It is essential to pay attention to how your child is feeling. Being grouchy, keeping to themselves, or not talking much might be signs of a JIA flare.If you sense something is wrong, reach out to your child's healthcare provider. Encourage your child to learn about JIA and speak up when they notice worsening symptoms. With time, they will learn to spot an incoming flare and let you know. Prescribed Treatment Medications used to treat JIA can reduce pain, improve function, and minimize the potential for joint damage.The medications used to help children with juvenile idiopathic arthritis can help decrease pain, improve function, and minimize potential joint damage.Medications used to treat JIA include:Disease-modifying antirheumatic drugs (DMARDs): DMARDs are used when NSAIDs alone do not help to manage a child's joint pain or if your child's provider feels there is a risk for joint damage. The most commonly prescribed DMARD for JIA is methotrexate.Biologic drugs: Some biologic drugs help to reduce systemic (body-wide) inflammation and prevent joint damage. Others work to suppress the immune system to stop inflammation in the first place. Biologic drugs include:Tumor necrosis factor alpha inhibitors (TNFi) like Humira (adalimumab), Enbrel (etanercept), Remicade (infliximab), Simponi (golimumab), and Cimzia (certolizumab pegol)Other biologic response modifiers (OBRM) like Orencia (abatacept), Actemra (tocilizumab), Kineret (anakinra), and Ilaris (canakinumab).Corticosteroids: Medications like prednisone can control inflammation and other JIA symptoms until longer-term medications take effect. They can also be used to manage severe disease flares. However, these drugs can interfere with growth and increase a child's risk of severe infections, so they should be taken for the shortest time possible.
Related: The 5 Main Classes of Rheumatoid Arthritis Drugs
In addition to medication, your child's healthcare provider might also recommend physical therapy. Physical therapy can help to:Improve and maintain range of motion in affected joints Prevent injuries from physical activityRelieve painStrengthen the muscles around joints Prognosis Into AdulthoodThe prognosis of JIA has improved over the past few decades due to the introduction of newer medications, including biologics. Healthcare providers previously believed children would outgrow the condition, but newer research shows that JIA can persist into adulthood.It is difficult for healthcare providers to predict which children will go into disease remission and which ones will continue to need treatment into adulthood.Some research shows children with oligoarticular JIA are more likely to achieve disease remission and discontinue all treatments compared to other JIA types. Early recognition and appropriate therapies help to improve outcomes. Children with most forms of JIA can have unaffected life expectancies. However, systemic JIA can lead to life-threatening complications. Such complications include:Macrophage activation syndrome (MAS) is an aggressive inflammatory response that overwhelms the body.Lung and heart problems include pulmonary artery hypertension and interstitial lung disease.Bone and joint damage: Persistent inflammation can slowly damage the joints, leading to reduced range of motion, function loss, and the need for joint replacement surgery. Chronic inflammation of the joint lining can also affect bone growth and development, leading to uneven arm or leg growth or slow overall growth.JIA can lead to adult-onset Still's disease, which is an adult form of systemic JIA. It is a rare type of inflammatory arthritis that can cause fevers, sore throat, joint pain, and rash that comes and goes quickly.
Can You Get Disability Benefits With JIA?Children with JIA may qualify for disability benefits if their JIA affects their mobility and function. But JIA would have to seriously limit the child's activities for them to qualify for Social Security disability. Disability benefits would also be determined based on the family's income and resources.
Follow-Ups and Ongoing Monitoring JIA requires regular care, including ongoing monitoring and treatment. When your child is seen regularly by a healthcare provider, the provider can monitor how treatments are working.The healthcare provider can also adjust treatments due to side effects if different dosages are needed and provide new treatments. They can also track remission through blood work and imaging. If treatments are working well, they may reduce or stop medicines. The most appropriate healthcare provider for treating JIA is a pediatric rheumatologist, a physician who specializes in childhood arthritis and other bone and joint problems affecting children. If you cannot find a pediatric rheumatologist in your area, you can work with your child's pediatrician and a rheumatologist. Additional specialists who can work with your child include:An occupational therapist who can teach ways to protect joints, manage pain, and perform activities of daily livingA physical therapist who can teach your child exercises to manage JIA, build strength, and restore flexibility of jointsA mental health professional to help your child cope with difficulties related to their medical condition An ophthalmologist who can diagnose and treat eye problems related to JIAAn orthopedist who can treat bone joint injuries or issues related to JIAA rheumatology nurse who can be a primary contact for your child's rheumatologist to discuss appointments, tests, medications, and more regarding your child's arthritis careA social worker or family advocate who can work with your child and family to manage changes related to arthritis and provide resources, including those related to school and learning challenges
Related: What Type of Doctor Treats Autoimmune Diseases?
Summary Juvenile idiopathic arthritis (JIA) is a type of arthritis that affects children. It is classified into six subtypes: oligoarticular JIA, polyarticular JIA, enthesitis-related arthritis, psoriatic arthritis, systemic onset JIA, and undifferentiated JIA. JIA causes joint pain, swelling, and stiffness. It may also cause systemic symptoms like fever and fatigue. The duration of the condition can vary, but it can last for months or years. In some cases, it may progress into adulthood.Treatments for JIA include NSAIDs, DMARDs, biologics, and corticosteroids. Physical and occupational therapy can also help.The prognosis for JIA depends on the type of JIA, but most types are manageable. Systemic onset JIA can be a severe condition, but long-term treatment can prevent complications. Most children with JIA can live happy and active lives and are not limited by their disease. If you suspect your child has JIA, contact their pediatrician right away. Early diagnosis and treatment can prevent joint damage and other disease complications. Read the original article on Verywell Health.
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