July is Juvenile Arthritis Awareness Month

Did you know that arthritis can also affect children?

It’s true — and that’s why Juvenile Arthritis Awareness Month is so important. Founded by the Arthritis Foundation, this campaign aims to raise awareness about this condition and share resources to help those affected by it. With kids, arthritis affects not just joints, but other organs too! Their immune system attacks their joints, causing swelling, stiffness, and sometimes permanent damage if left untreated.

History of Juvenile Arthritis Awareness Month

Even though the name ‘arthritis’ itself relates to the joints — it means ‘joint inflammation’ in Latin — juvenile arthritis can affect the eyes, skin, and the gastrointestinal tract. The term was first described in the 18th century by pediatrician George Frederick Still. Acceptance for this illness took much longer, however, and it was only until a century later that a specialized center for juvenile rheumatism was established in Berkshire. The center’s original purpose was to treat people afflicted with rheumatic fever, which was prevalent in the years following the Second World War. As hygiene and nutrition improved, this disease declined. Soon, the center turned its attention toward children and juvenile arthritis, which was known as Still’s Disease back then. Dr. Barbara Ansell from the center was one of the first to recognize that the development of the disease, its progression, and response to treatment was different for different people. This unit operated for 39 years and has greatly contributed to the causes and outcomes of juvenile arthritis treatment we see today.

Over the next century, such centers were established in Germany, too, but recognition from major organizations like the American Rheumatism Association (ARA) and the International League Against Rheumatism (ILAR) occurred later in the 19th century. The fact that this is a relatively rare illness, fewer physicians specialize in it, and pharmaceutical companies largely target adult markets means that therapeutic advancements in juvenile arthritis have been historically slow. The treatment gradually shifted from physical — physiotherapy and hydrotherapy, splinting, and surgery — to a drug-based regimen. Recent American history shows the Food and Drug Administration has supported many incentives to drive U.S. pharmaceutical agencies to include children, too, like their Best Pharmaceuticals for Children Act (BPCA) and Pediatric Research Equity Act (PREA).

Overview of Juvenile Idiopathic Arthritis (JIA)

Like adults, children can develop arthritis. The most common type of chronic or long-lasting, arthritis that affects children is called juvenile idiopathic arthritis (JIA). JIA broadly refers to several different chronic disorders involving inflammation of joints (arthritis), which can cause joint pain, swelling, warmth, stiffness, and loss of motion. The various forms of JIA have different features, such as the pattern of joints involved and inflammation of other parts of the body besides the joints. JIA may last a limited time, such as a few months or years, but in some cases, it is a lifelong disease that requires treatment into adulthood.

JIA is “idiopathic,” meaning that its origins are not understood. While the exact causes of JIA are unknown, it begins when the immune system becomes overactive and creates inflammation.

With treatment, most children achieve periods of wellness (remission), and sometimes the disease goes away permanently with no further need for medications. It is important to see a doctor early if your child has swollen or stiff joints because delaying therapy can lead to joint damage, a lesser response to treatment, and other problems.

Who Gets Juvenile Idiopathic Arthritis?

By definition, JIA begins in children and adolescents before the 16th birthday. Most types of the disease are more frequent in girls, but enthesitis-related JIA, a form of the disease that involves inflammation of the places where ligaments and tendons (flexible bands of tissue) attach to bones, is more common in boys. Systemic JIA, a rare type of JIA that features fever and rash, affects boys and girls equally. Children of all races and ethnic backgrounds can get the disease.

It is very rare for more than one member of a family to have JIA, but children with a family member with chronic arthritis, including JIA, are at a slightly increased risk of developing it. Having a family member with psoriasis is a risk factor for a form of JIA called psoriatic JIA.

Types of Juvenile Idiopathic Arthritis

There are multiple types of JIA, each with distinct features. Generally, they all share arthritic symptoms of joint pain, swelling, warmth, and stiffness that last at least 6 weeks. The types are:

Oligoarticular juvenile idiopathic arthritis

This is the most common and mildest form, affecting four or fewer joints. It is considered persistent if symptoms continue for 6 months or longer, and extended if five or more joints become involved after 6 months of illness. Commonly affected joints are knees or ankles. A form of eye inflammation called chronic (long-lasting) uveitis can develop in children with this form of JIA. About half of children with JIA have this type.

Polyarticular juvenile idiopathic arthritis–rheumatoid factor negative

This is the second most common type, affecting five or more joints in the first 6 months. Tests for rheumatoid factor are negative. The rheumatoid factor blood test checks for autoimmune disease, especially rheumatoid arthritis, which is an adult form of arthritis. Some of these children develop chronic uveitis.

Polyarticular juvenile idiopathic arthritis–rheumatoid factor positive

A child with this type has arthritis in five or more joints during the first 6 months of the disease. Tests for rheumatoid factor, a marker for autoimmune disease, are positive. It tends to occur in preteen and teenage girls, and it appears to be essentially the same as adult rheumatoid arthritis.

Enthesitis-related juvenile idiopathic arthritis

This form of JIA involves both arthritis and enthesitis. Enthesitis happens when inflammation occurs where a ligament or tendon attaches to a bone. The most common locations for enthesitis are the knees, heels, and bottoms of the feet. Arthritis is usually in the hips, knees, ankles, and feet, but the sacroiliac joints (at the base of the back) and spinal joints can also become inflamed. Some children get episodes of acute anterior uveitis, a sudden onset of inflammation of the front of the eye. Unlike most other forms of JIA, enthesitis-related JIA is more common in boys.

Psoriatic juvenile idiopathic arthritis

Children with this type have psoriasis, a skin condition, as well as inflammation of the joints. The skin condition usually appears first, but sometimes painful, stiff joints are the first sign, with the skin disease occurring years later. Pitted fingernails and dactylitis (swollen fingers or toes) are also signs of the disease.

Systemic juvenile idiopathic arthritis

Systemic means the disease can affect the whole body, not just a specific organ or joint. Systemic JIA usually starts with fever and rash that come and go over the span of at least 2 weeks. In many cases, the joints become inflamed, but sometimes not until long after the fever goes away, and sometimes not at all if treatment is started quickly. In severe forms, inflammation can develop in and around organs, such as the spleen, lymph nodes, liver, and linings of the heart and lungs. Systemic JIA affects boys and girls with equal frequency.

Undifferentiated arthritis

This category includes children who have symptoms that do not fit into any of the other types or that fit into more than one type.

Symptoms of Juvenile Idiopathic Arthritis

Symptoms of JIA vary depending on the type, but all forms share persistent joint pain, swelling, warmth, and stiffness that are typically worse in the morning and after a nap or prolonged sitting. The pain may limit movement of the affected joint, although many children, especially younger ones, will not complain of pain. One of the earliest signs may be limping in the morning due to disease in one or both legs.

The symptoms of JIA may go through cycles, flaring for a few weeks or months followed by periods when they go into remission. Some children have just one or two flares and never have symptoms again, while others have many flares or symptoms that never fully go away. Besides joint problems, the inflammation associated with JIA can cause other symptoms, such as:

Eye Inflammation

Uveitis (inflammation of the front and middle parts of the eye) often occurs in children with JIA. It usually starts within a few years after the JIA diagnosis, but in a small fraction of children, it appears before the joint symptoms or many years afterward. The type of JIA a child has may cause the development of different types of uveitis. Children with: 

• Oligoarticular JIA, especially when it begins at an early age, can have chronic (long-lasting) uveitis that has mild or no symptoms. Children with polyarticular JIA without rheumatoid factor, or with psoriatic JIA are also at risk.
• Enthesitis-related JIA and some children with psoriatic JIA can have episodes of acute anterior uveitis, which has a sudden onset and causes eye pain, eye redness, and sensitivity to light.

If left untreated, uveitis can lead to eye problems such as cataracts, glaucoma, and vision loss, so it is important for children with JIA to have a frequent eye exam

Skin Changes

Depending on the type of JIA a child has, he or she may develop skin changes. Children with:

Systemic JIA who have fevers can get a light red or a pink rash that comes and goes.

• Psoriatic JIA can develop scaly red patches of skin. Psoriatic JIA can also cause pitted nails and dactylitis (swollen fingers or toes).
• Polyarticular JIA with rheumatoid factor can get small bumps or nodules on parts of the body that receive pressure, such as from sitting.

Fever

Patients with systemic JIA typically have daily fevers when the disease begins or flares. The fever usually appears in the evening, and the rash may move from one part of the body to another, usually happening with the fever. Patients with other types of JIA do not generally develop fevers.

Growth Problems

Inflammation in children with any type of JIA can lead to growth problems. Depending on the severity of the disease and the joints involved, bones near inflamed  joints may grow too quickly or too slowly. This can cause one leg or arm to be longer than the other or can result in a small or misshapen chin. Overall growth also may be slowed if the disease is severe. Growth normally improves when inflammation is well-controlled through treatment.

What Causes Juvenile Idiopathic Arthritis?

In JIA, the body’s immune system—which normally helps to fight off infections and heal cuts and wounds—mistakenly attacks some of its own healthy cells and tissues. The result is inflammation, marked by pain, swelling, warmth, and stiffness. Inflammation from JIA can damage the joints, eyes, or other affected organs.

Scientists do not know why the immune system attacks healthy tissues in children with JIA, but they believe that a complex mix of genes and environmental factors are involved.

While the origin of the immune system’s overreaction in JIA is unknown, scientists have identified some of the molecules that contribute to inflammation in some forms of the disease. We now know that three molecules—TNF-alpha, IL-6, and IL-1—are involved in creating inflammation in the joints of many children with JIA. This has led to new therapies that specifically target these molecules.

JIA By the Numbers

Juvenile Idiopathic Arthritis (JIA) is the most common childhood chronic rheumatologic disorder. There is a yearly incidence of 2-20 cases / 100,000 individuals with a prevalence of 16-50 cases / 100,000 individuals, meaning there is an estimated total of 294,000 children affected in the United States (Praken, 2011). Prevalence can vary with ethnicity and is more common in northern Europeans (Eisenstein, 2014).

Observing Juvenile Arthritis Awareness Month

Read and learn about juvenile arthritis

Learn more about juvenile arthritis, the types of arthritis that affect children, what are the common treatments available, and what you can do to support this cause this month. You can get more information on the Arthritis Foundation website, which provides resources for parents, kids, and teens to manage medication, activity, and eating habits.

Raise Awareness

The key to a successful cause is awareness. Go online with your story and share what you have learned about Juvenile Arthritis Awareness Month. Encourage others to observe this month, too. If you don't prefer social media for sharing your thoughts, make sure at least one other person knows about juvenile arthritis.

Host a Community Event

All through this month, the Arthritis Foundation runs conferences and camps for parents and children. You can talk to your community leaders about hosting similar events at the local pediatric facilities, taking inspiration from the above-mentioned website. Invite guest speakers or talk to those willing to share their fight with juvenile arthritis.

Sources:

http://blog.arthritis.org/juvenile-arthritis/

https://www.niams.nih.gov/community-outreach-initiative/health-observances

https://www.niams.nih.gov/health-topics/juvenile-arthritis

https://www.niams.nih.gov/health-topics/kids/healthy-joints

https://nationaltoday.com/juvenile-arthritis-awareness-month/