Juvenile Idiopathic Arthritis (JIA)

 JIA is the most common type of arthritis in kids and teens.

Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in kids and teens. It typically causes joint pain and inflammation in the hands, knees, ankles, elbows and/or wrists. But, it may affect other body parts too . JIA used to be called juvenile rheumatoid arthritis (JRA), but the name changed because it is not a kid version of the adult disease. The term “juvenile arthritis” is used to describe all the joint conditions that affects kids and teens, including JIA.

Causes
JIA types are autoimmune or autoinflammatory diseases. That means the immune system, which is supposed to fight invaders like germs and viruses, gets confused and attacks the body’s cells and tissues . This causes the body to release inflammatory chemicals that attack the synovium (tissue lining around a joint). It produces fluid that cushions joints and helps them move smoothly. An inflamed synovium may make a joint feel painful or tender, look red or swollen or difficult to move.  

The word “idiopathic” means unknown, and researchers aren’t sure why kids develop JIA. They believe kids with JIA have certain genes that are activated by a virus, bacteria or other external factors. But there is no evidence that foods, toxins, allergies or lack of vitamins cause the disease.


 

Symptoms


It’s also possible that a child may start off with one type of JIA but develop symptoms of another type later. 
The most common symptoms include:

  • Joint pain or stiffness; may get worse after waking up or staying in one position too long.
  • Red, swollen, tender or warm joints.
  • Feeling very tired or rundown (fatigue).
  • Blurry vision or dry, gritty eyes.
  • Rash.
  • Appetite loss.
  • High fever.

Certain symptoms are specific to the type of arthritis a child has. There are six types of JIA:

  • Oligoarthritis: Affects four or fewer joints, typically the large ones (knees, ankles, elbows). Most common subtype of JIA.
  • Polyarthritis: Affects five or more joints, often on both sides of the body (both knees, both wrists, etc.). May affect large and small joints. Affects about 25% of children with JIA.
  • Systemic: Affects the entire body (joints, skin and internal organs). Symptoms may include a high spiking fever (103°F or higher) that lasts at least two weeks and rash. Affects about 10% of children with JIA.
  • Psoriatic arthritis (PsA): Joint symptoms and a scaly rash behind the ears and/or on the eyelids, elbows, knees, belly button and scalp. Skin symptoms may occur before or after joint symptoms appear. May affect one or more joints, often the wrists, knees, ankles, fingers or toes. 
  • Enthesitis-related: Also known as spondyloarthritis. Affects where the muscles, ligaments or tendons attach to the bone (entheses). Commonly affects the hips, knees and feet, but may also affect the fingers, elbows, pelvis, chest, digestive tract (Crohn’s disease or ulcerative colitis) and lower back (ankylosing spondylitis). More common in boys; typically appears in children between the ages of eight and 15. 
  • Undifferentiated: Symptoms don’t match up perfectly with any of the subtypes, but inflammation is present in one or more joints.  

JIA symptoms may also come and go. Periods of lots inflammation and worsening symptoms are called flares. A flare can last for days or months.
 

Health Effects
If JIA inflammation goes unchecked, it can damage the lining that covers the ends of bones in a joint (cartilage), and the bones themselves. Here are some other ways JIA can affect the body:
  • Eyes. Dryness, pain, redness, sensitivity to light and trouble seeing properly caused by uveitis (chronic eye inflammation). More common with oligoarthritis.
  • Bones. Chronic inflammation and use of corticosteroids may cause growth delay in some children with JIA. Bones may get thinner and break more easily (osteoporosis).
  • Mouth/Jaw. Difficulty chewing, brushing or flossing. More than half of children with JIA have jaw involvement. 
  • Neck. Inflammation of the cervical spine can cause neck pain or stiffness. Swollen neck glands could also signal an infection for kids with SJIA or who take immunosuppressing drugs.  
  • Ankles/feet. Foot pain and difficulty walking. More common in polyarthritis and enthesitis-related arthritis. 
  • Skin.  Symptoms can range from a faint salmon colored rash (SJIA) to a red, scaly rash (psoriatic arthritis).  
  • Lungs.  Inflammation and scarring that can lead to shortness of breath and lung disease. May occur in SJIA.
  • Heart. Inflammation may cause damage to the heart muscle. May occur in SJIA.
  • Digestive Tract. Abdominal pain and diarrhea. More common in children with spine arthritis or ankylosing spondylitis. 
  • Reproductive organs. Late onset of puberty. Certain drugs such as cyclophosphamide may lead to fertility problems later. 
  • Weight loss or gain. Due to changes in appetite, jaw involvement or difficulty exercising. Being overweight puts extra stress on the joints. 

Controlling inflammation and managing disease can prevent damage and complications from these health effects. 
 
Diagnosis
According to American College of Rheumatology (ACR) a child must have inflammation in one or more joints lasting at least six weeks, be under 16 years old and have all other conditions ruled out before being diagnosed with JIA.  

A pediatrician may be the first doctor to start figuring out what’s causing symptoms. It’s likely that parents will be referred to a rheumatologist (a doctor with specialized training in treating arthritis). Some rheumatologists only treat children. Others only treat adults. Some of them treat both. A medical history, physical examination and blood tests helps to make the correct diagnosis. 

Medical history. The doctor will ask questions about the child’s health history, when symptoms started and how long they lasted. This helps rule out other causes like trauma or infection. The doctor will also ask about the family's medical history.

Physical examination. The doctor will look for joint tenderness, swelling, warmth and painful or limited movement and test range of motion.  Eyes and skin may also be checked. 

Laborator tests. The doctor may order blood tests that look for certain proteins and chemicals found in some people with arthritis. These tests include:
  • Erythrocyte sedimentation rate (ESR, or “sed rate”) and C-reactive protein (CRP) tests: High ESR rates and CRP levels signal severe inflammation in the body.  
  • Antinuclear antibody (ANA) test: A positive ANA test is associated with many types of arthritis, but kids without JIA may also have a positive ANA. 
  • Rheumatoid factor (RF) test: May show up in children with polyarthritis. A positive rheumatoid factor may signal more serious disease.
  • HLA-B27 typing (a genetic marker): The HLA-B27 gene is associated with enthesitis-related types of arthritis, such ankylosing spondylitis. 
  • Complete blood count (CBC): Raised levels of white blood cells and decreased levels of red blood cells is linked to certain types of arthritis.
  • Imaging. The doctor may order imaging tests, such as X-rays, ultrasound and MRI or CT scans, to look for signs of joint damage. 
Treatment
There is no cure for JIA but remission (little or no disease activity or symptoms) is possible.  Early aggressive treatment is key to getting the disease under control as quickly as possible. 

The goals of JIA treatment are to:
  • Slow down or stop inflammation. 
  • Relieve symptoms, control pain and improve quality of life.
  • Prevent joint and organ damage.
  • Preserve joint function and mobility. 
  • Reduce long-term health effects.
  • Achieve remission (little or no disease activity or symptoms).


Treatment for JIA varies depending on disease type and severity. A well-rounded plan includes medication, complementary therapies and healthy lifestyle habits.  
Medications

Drugs that control disease activity

Disease-modifying antirheumatic drugs (DMARDs). These drugs work to modify the course of the disease. DMARDs relieve symptoms by suppressing the immune system so it doesn’t attack the joints.  

•   Traditional DMARDs. These have been used the longest and have a broad immune-suppressing effect. The most commonly-used drug for JIA is methotrexate. These medicines are available in pill or injection form. 
•   Biologics. These drugs target certain steps or chemicals in the inflammatory process and may work more quickly than traditional DMARDs. They are self-injected or given by infusion in a doctor’s office.
 

Drugs that relieve symptoms

Nonsteroidal anti-inflammatory drugs (NSAIDs) and analgesics (pain relievers). These drugs relieve pain but cannot reduce joint damage or change the course of JIA. These medications are available over-the-counter or by prescription.  

Every child with JIA is different. The standard of care involves trying methotrexate first, but many doctors start with a biologic/DMARD combination to combat inflammation as quickly as possible. As doctors monitor the disease, drugs may be added or removed.

For more information on JIA drugs, visit arthritis.org/drugguide.

Surgery

Thanks to treatment advances, including biologics, many children will never need surgery. But for children whose disease couldn’t be controlled early enough, surgery can provide much needed relief and restore joint function. 

Damaged parts of a joint are replaced with metal, ceramic or plastic prosthesis. Hip and knee replacements are most common, and many surgeries can be performed on an outpatient basis. There are other surgeries that can improve joint function and quality of life but require much less cutting than joint replacement. For example, with arthroscopy, a thin, lighted tube with a camera attached is inserted through a small incision. This helps the surgeon examine the child’s joints and perform procedures, such as removing a loose piece of cartilage. An orthopedic surgeon will evaluate and determine if surgery is the best option. 
 

Nondrug Therapies

Exercise
Regular exercise helps ease joint stiffness and pain. Low-impact and joint-friendly activities like walking, swimming, biking and yoga are best, but kids with well-controlled disease can participate in just about any activity they wish, if their doctor or physical therapist approves. 

Physical Therapies and Assistive Devices

Physical and occupational therapy can improve a child’s quality of life by teaching them ways to stay active and how to perform daily tasks with ease. Children with JIA may also have trouble with balance and weaker motor skills, or the ability to move and coordinate large muscle groups. Participating in regular physical and occupational therapy can improve coordination and balance, among other things. Here are some other ways physical and occupational therapists can help a child with JIA:
•    Teach and guide them through strengthening and flexibility exercises. 
•    Perform body manipulation.
•    Prescribe assistive devices (e.g. braces, splints).
Self-Care
Certain habits can help manage disease and relieve symptoms.  

Healthy Eating

While there is no special JIA diet, studies show that some foods help to curb inflammation. These include the foods found in the Mediterranean diet, i.e. fatty fish, fruits, vegetables, whole grains and extra virgin olive oil among others. Children with JIA should avoid or cut back on foods that can cause inflammation such as high-fat, sugary and processed foods. 

Balancing Activity With Rest

When JIA is active, and joints feel painful, swollen or stiff, it’s important to balance light activity with rest. Rest helps reduce inflammation and fatigue that can come with a flare. Taking breaks throughout the day protects joints and preserves energy.

Hot and Cold Treatments

Heat treatments, such as heat pads or warm baths, work best for soothing stiff joints and tired muscles. Cold is best for acute pain. It can numb painful areas and reduce inflammation.

Topical Products

These creams, gels or stick-on patches can ease the pain in a joint or muscle. Some contain the same medicine that’s in a pill, and others use ingredients that irritate nerves to distract from pain. 

Mind-Body Therapies

There are different ways to relax and stop focusing on pain. They include meditation, deep breathing and practicing visualization, or thinking about peaceful places or happy memories. Children with JIA may also benefit from certain distraction techniques to lessen pain, especially during shot time. These include listening to music, coloring or drawing, reading and being read to. 

Massage and acupuncture can also help reduce pain and ease stress or anxiety. Acupuncture involves inserting fine needles into the body along special points to relieve pain. If there’s a fear of needles, acupressure, which uses firm pressure, may be used instead.



 

Supplements

The use of supplements is rarely studied in children. But some supplements that adults take may help children too. These include curcumin, a substance found in turmeric, and omega-3 fish oil supplements, which may help with joint pain and stiffness. Taking calcium and vitamin D can help build strong bones. Always discuss supplements and vitamins with a child’s doctor. Some may cause  side effects and interact with other medications. 

Stress and Emotions

Children and teens with JIA are more likely to get depressed because they are living with a chronic disease. Having a strong support system of friends and family can provide emotional support during tough times. Children with JIA can make new friends dealing with similar struggles at various Arthritis Foundation JA events held throughout the year. Teens may also participate in the Foundation’s iPeer2Peer program, which matches a young adult mentor with arthritis to a teen with the disease. Therapist and psychologists can also help kids with JA deal with tough emotions and teach positive coping strategies. 
 

60%

Children with JIA who have oligoarthritis; mostly girls.

10%

Children with JIA have SJIA; boys and girls equally.

25%

Children develop uveitis within four years after diagnosis

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