Juvenile Idiopathic Arthritis
What is Juvenile Idiopathic Arthritis?
Juvenile Idiopathic Arthritis (JIA) consists of a group of childhood chronic (long term) arthritis including what has been formerly called Juvenile Rheumatoid Arthritis (JRA) and Juvenile Chronic Arthritis (JCA). It now also includes other forms of chronic childhood arthritis such as psoriatic arthritis, enthesitis-related arthritis and undifferentiated arthritis. JIA is an autoimmune disease, which is defined by the presence of arthritis for at least six weeks in a child less than seventeen years of age. Arthritis is joint inflammation, which may cause joint swelling, pain and/or stiffness.
JIA is the most common cause of chronic arthritis in children and affects children worldwide, with no racial predilection. Approximately 1/1,000 children, or more than 300,000 children in the U.S. have JIA. Although children of all ages may get JIA, it is rare before 6 months of age. Arthritis may affect any joint, even your jaw and spine. JIA may be categorized into several subtypes, some of which may be associated with other diseases, which may affect the eyes or gastrointestinal tract, for example. Although there are several sub-types of JIA, all patients are affected by chronic arthritis. The severity of arthritis is variable, and patients may have an unpredictable flare (worsening) of their arthritis. Sometimes this flare may relate to the weather, viral infections or inactivity.
How is JIA diagnosed?
There is no specific blood test that can diagnose JIA, which is why the diagnosis is based primarily on history and physical exam. Children may complain of pain, limp, reluctance to move a limb or stiffness, but often children may have arthritis with joint swelling and minimal pain, thereby often delaying the diagnosis. There are some blood tests, such as an antinuclear antibody (ANA), rheumatoid factor (RF) or anti-cyclic citrullinated peptide (CCP), which may support the doctor’s suspicion for arthritis. Sometimes the doctor may request images, such as x-rays or an MRI, in order to further define the arthritis, or check to see if there is some other cause for the joint pain.
What causes JIA?
As suggested by the accepted terminology of “idiopathic” arthritis, there is no clear cause of JIA. Although there is often some minor trauma (e.g. fell off the swing) before the physician or parent notices the arthritis, this is not considered to be a causative factor. Genetic, hormonal, environmental and infectious factors may be involved, although no clear trigger has been consistently identified. As JIA is an autoimmune disease that may be characterized by the presence of autoantibodies, such as an ANA, an unfortunate combination of these unknown factors in certain genetically susceptible children leads to an abnormal and overactive immune system that results in painful, swollen joints i.e. arthritis.
Subsets of Juvenile Idiopathic Arthritis
Oligoarticular Juvenile Idiopathic Arthritis
Oligoarticular JIA (oligo-JIA) is the most common form of JIA, and involves arthritis in 4 or fewer joints. Approximately 60% of patients with JIA have this form of chronic arthritis. Girls are affected more frequently than boys. Patients with oligo-JIA are at risk of developing an inflammatory eye disease called uveitis. Children with uveitis (yoo-vee-eye-tis) frequently do not complain of anything, and therefore requiring regular eye (slit lamp) exams. The patients at greatest risk are young children, early in their diagnosis with a positive ANA blood test. There is also subset of oligo-JIA patients that progress to have arthritis involving more than 5 joints, called extended oligo-JIA.
Polyarticular Juvenile Idiopathic Arthritis
Polyarticular Juvenile Idiopathic Arthritis (Poly-JIA) is a less common subset of JIA, which involves arthritis in many (5 or more) joints, often with finger involvement. Although young children may also have this form of arthritis, teenagers with poly-JIA and a positive rheumatoid factor blood test have an arthritis that resembles adult rheumatoid arthritis. Poly-JIA is a very intense arthritis that requires the use of medicines, such as Disease-Modifying-Anti-Rheumatic-Drugs (DMARDs) or biologics in order to treat the arthritis and prevent joint damage, deformity and disability. Many children may have other symptoms related to their arthritis, such as morning stiffness, fatigue, poor appetite (with possible weight loss) and anemia. These children may also have blood tests that reveal inflammation, with elevated inflammatory markers, such as an ESR or CRP.
Systemic Onset Juvenile Idiopathic Arthritis
Systemic Onset Juvenile Idiopathic Arthritis (So-JIA) is another subtype of JIA affecting 10% of children with JIA. It is characterized by the presence of high daily fevers, a “salmon-colored” rash that is usually worse with fever, and arthritis. Sometimes the arthritis is not initially present when the children have fever and rash. Children with So-JIA are often quite ill, with weight loss, enlarged lymph nodes and anemia. Some children may develop effusions (fluid) around their heart and lungs, liver disease, which may rarely progress to potentially life-threatening disease with severe bleeding, as well as brain and bone marrow malfunctioning. Many children with So-JIA require steroids to control their disease in addition to DMARDs and biologics.
Psoriatic Arthritis
Psoriatic Arthritis is another subtype of JIA in which a child has chronic arthritis and a skin rash called psoriasis, which often looks like a dry, silvery, scaly rash. There may not be psoriasis at the time the arthritis is diagnosed, but there may be a strong family history of psoriasis.
Enthesitis-related Arthritis
Another subtype of JIA is Enthesitis-related arthritis, which may also be called Spondyloarthropathy. In addition to chronic arthritis, these children have enthesitis, which is inflammation of structures, such as ligaments, around the joints, which may cause pain. Many of these patients may also have arthritis of the back and neck, as well as morning stiffness. Some children with Enthesitis-related Arthritis may also develop inflammation of their gastrointestinal system.
How do you treat JIA?
Overview
Today, most children with JIA can live long, healthy lives with full physical functioning, attending school and playing sports. Unfortunately, some children may require a significant amount of medication, and continue to have arthritis for many years. In general, the goal is to diagnose children with JIA early, and treat appropriately intensively, in order to minimize joint damage and prevent disability. The doctor must tailor the treatment to each individual patient. It is essential that there is a healthy and open communication between the doctor and family in order to establish trust and effectively treat the child with JIA. Many specialists may be required in the treatment of a child with JIA, forming the therapeutic team. In addition to the rheumatologist and pediatrician, this team may include members such as physical therapists, social workers, teachers, psychologists, ophthalmologists (eye doctors), nutritionists, orthopedic surgeons, dentists and others.
Medications
In general, children with JIA are treated with similar medications as adult rheumatoid arthritis. Of course, when prescribing medications the doctor always takes into consideration the physical and psychosocial effects of medicine on a growing child, as well as the fact that many children require liquid preparations. Most children with arthritis are treated with a type of non-steroidal anti-inflammatory drug (NSAID), such as naproxen (Naprosyn) or ibuprofen (like Motrin or Advil). Disease-Modifying-Anti-Rheumatic-Drugs (DMARDs) may be necessary in certain patients, such as in polyarticular or systemic JIA. Commonly used DMARDs include methotrexate (Rheumatrex) and sulfasalazine (Azulfidine) among others. Biologics are newer agents used in the treatment of arthritis, which target specific cells or chemicals involved in arthritis. Commonly used biologics include the anti-TNF biologics, including etanercept (Enbrel), infliximab (Remicade) and adalimumab (Humira). This is an area of intense research, and newer agents are also available such as rituximab (Rituxan), abatacept (Orencia) and anakinra (Kineret), among other agents in development. All of these newer medications are administered via subcutaneous injections or intravenous infusions. Steroids, such as prednisone, may also be required in certain patients with polyarticular or systemic JIA. Many rheumatologists may recommend various combinations of these medications to effectively treat JIA.
Joint Injections
Steroid joint injections are the ideal treatment for children with Oligo-JIA with only 1 or 2 joints affected. A joint injection is a quick, simple and long-lasting technique to instill medication directly into the joint, and avoid the systemic side effects and technical difficulties of daily medication in a young child.
Physical and Occupational Therapy
Physical and/or occupational therapy are often required for children with JIA. The focus of therapy should include pain control, improvement of range of motion and muscle strengthening in order to prevent deformity and disability, with the ultimate goal of full physical functioning. Therapists may recommend splints/braces, warm or cold treatments, and home exercise programs among other treatments. Both a motivated therapist and patient/family are essential for success.
Radiology
Your doctor may take x-rays of the affected joints to check for any erosion (bone destruction), as well as the overall health of the bones. Sometimes your doctor may order an MRI of the joint as well. Some doctors may repeat these exams periodically in order to monitor treatment.
Laboratory Testing
Regular laboratory testing may be necessary to monitor disease activity, such as anemia and inflammatory markers. Doctors may also need to monitor the child for any side effect of the medication he/she is taking.
Ophthalmology
Depending on the subtype of arthritis, patients with JIA should see the ophthalmologist (eye doctor, M.D. or D.O.) anywhere from every three months to once a year. It is important that the ophthalmologist perform a slit lamp exam to check for uveitis at each visit. Patients must go to see the eye doctor even if their eyes are not bothering them, since children can commonly have serious eye disease without any complaints.
Surgery
Children are being diagnosed with JIA much earlier than in the past as a result of increased awareness. There have also been many advances in the treatment of arthritis with newer medications. Both of these facts have resulted in fewer children needing orthopedic surgery. Nevertheless, there are some children who may require surgery to alleviate pain and/or correct deformity or disability as a result of arthritis.
School
Regular attendance at school is a MUST, for almost all children with JIA. No child with JIA may discriminated against because they have arthritis, and public schools are required to provide accommodations for the child by law, according to the Federal Act 504. Your doctor may provide the school with certain guidelines that may be helpful to everyone. It is important for normal psychosocial development that children with JIA interact with their peers at school. Home schooling because of JIA is only rarely appropriate.
Diet and Exercise
Children with arthritis need to move their bodies in order to maintain their physical conditioning, using a “move it, or lose it” philosophy. If a child is having a flare of their arthritis, the child may do a different activity. For example, a child may do jumping jacks instead of push-ups in gym if they have a flare of wrist arthritis. The gym teacher must provide modified physical education.
All children with JIA need to eat a “healthy diet”, minimizing their junk food and other high-calorie foods. Some children with JIA may be taking prednisone, which makes people very hungry and put on weight very easily. The extra weight is not good for your bones and joints. Healthy snack alternatives should always be an arm’s length away.
Assistance programs for children with JIA
There are federal and state programs that are designed to provide accommodations for children with JIA at home and school, as well as family support services. Through these programs, such as a 504 Plan, children should be able to receive services, such as physical therapy, at school. There are also several private organizations that also provide support for families of children with JIA. Through these organizations, one may discover family support groups, as well as activities, such as summer camp that may be available to children with JIA.