Juvenile Rheumatoid Arthritis

Juvenile rheumatoid arthritis (JRA) – also known as juvenile idiopathic arthritis (JIA) – is an inflammation of one or more joints, with an onset of symptoms occurring in a child or adolescent aged 16 years or younger. To be classified as JRA, the condition must persist for a minimum of six weeks and all other possible causes must be excluded. 1

Although the exact causes of JRA remain unclear, it is known that the condition is an autoimmune disorder. This means that disordered functioning of the body’s immune system plays a role in the development of JRA.

In the majority of cases, the condition can be managed through prolonged treatment. This will typically involve a combination of medication and physical therapy. 2

There are various types of JRA, each with varying symptoms and levels of severity. 3

Oligoarthritis: The most common type of JRA, affecting roughly one third of those with the condition. For diagnosis, no more than four joints in a person’s body will be affected within the first six months of developing this condition. Oligoarthritis typically affects one or both knees, and can also cause chronic eye inflammation in some cases.

Polyarthritis: The second most common form of JRA, which can develop suddenly or steadily over a period of weeks. For diagnosis, five or more joints must be affected during the first six months of the condition developing. It may cause painful inflammation of the wrists, ankles, toes, hips, knees, neck and jaw.

Enthesitis-related JRA: This condition typically causes inflammation of the leg joints and spine, at sites where tendons or ligaments insert into joints, such as the heel. In some cases, this will be accompanied by acute uveitis (a painful eye condition). Enthesitis-related JRA is often associated with inflammatory bowel disease (IBD).

For more information about conditions related to inflammatory bowel disease (IBD), see these resources on its two main forms, Crohn’s disease and ulcerative colitis.

Psoriatic arthritis: A combination of a skin rash, caused by a long-lasting autoimmune disease of the skin called psoriasis, with joint pain, usually in the fingers and toes.

Systemic JIA: Also known as Still’s disease, this condition consists of joint pain combined with fever, tiredness, loss of weight, lack of appetite and a rash. It may lead to enlarged glands in the groin, arms and neck, as well as an enlarged spleen and liver.

Some of the most common JRA signs and symptoms include: 4

• Tenderness and/or swelling of the joints
• Joints that are warm to the touch
• Rash
• Fever
• Limping
• General pain
• Stiffness (especially following sleep or inactivity)
• Fatigue
• Sleep problems
• Swollen lymph nodes
• Weight loss and a reduced appetite

Symptoms typically vary from person to person although some are specific to certain subtypes of JRA. It is also possible for symptoms to alter over time, even over the course of a single day.

If symptoms arise it is important to seek medical attention. By examining the symptoms a child is experiencing and ruling out other possible causes, a doctor will generally be able to identify whether JRA is present. In addition, the free Ada app can be used to carry out a symptom assessment.

With prompt and appropriate treatment, the prognosis for JRA can be greatly improved. However, without treatment or if the condition is particularly severe, complications can arise.

Complications associated with JRA can include: 5

• Anemia (a lack of red blood cells or hemoglobin)
• Permanent joint damage
• Disproportionate limbs
• Stunted growth (caused by the condition as well as some medications used to treat it)
• Eye inflammation (typically painful and can in very severe cases lead to blindness)

Although complications are possible, the risk of them occurring can be reduced through careful management of the condition.

The causes of JRA are not fully understood. However, the condition is generally thought to be linked to a dysfunction of the immune system combined with genetic and/or environmental factors.

Research suggests that JRA may be triggered by abnormal immune responses to certain viruses or other agents, causing joint inflammation when it is not needed. Although it is uncommon for two members of the same family to experience JRA, genetic mechanisms are thought to play a role in determining who develops the condition.

Some research suggests human leukocyte antigens – a type of molecule which plays an important role in deciding how the body reacts to foreign agents – are particularly relevant to this process, with people who have an increased amount of a specific type of the molecule being at greater risk of developing JRA. 6 7

To be classified as JRA, the signs and symptoms of the condition must occur before the age of 16. These signs and symptoms are not always obvious or detectable, and as a child may not understand what is and isn’t normal, they may not complain about certain symptoms, such as joint pain.

However, by being aware of the symptoms of the condition and through careful monitoring of a child’s development, it should be possible for caregivers to identify the warning signs and ensure the required medical attention is sought.

There is no single test that can be used to diagnose JRA. Instead, the condition is diagnosed by ruling out other possible causes of the symptoms. This is done by using a combination of methods, including looking at an individual’s family history, carrying out a physical examination, blood tests and imaging scans. 8

Although it is rare for two or more members of the same family to be diagnosed with JRA, it is still important for doctors to look at one’s family medical history for any signs of predisposition to the condition. Doctors will pay particular attention to whether any family members have experienced an autoimmune disorder of any kind, as this may increase the chances of JRA.

The purpose of a physical examination is to look for the various early signs and symptoms of JRA that may be being experienced.

Doctors will look for signs and symptoms including:

• A limp
• Not using or a reluctance to use a certain limb
• Stiffness (especially in the morning or after inactivity)
• A fever which comes and goes
• Joint swelling (lasting for six weeks or more)
• Fatigue and inactivity
• Loss of motor skill function

A combination of different blood tests can be used to help diagnose JRA. These include:

• Rheumatoid factor: Generally the first test to be carried out. It tests for the rheumatoid factor antibody (an immune system molecule in the blood) which, although undetectable in more than 50% of cases, can indicate the presence of JRA.
• Erythrocyte sedimentation rate (ESR) A test to measure levels of inflammation in the body.
• C-Reactive protein: Another test that can measure levels of inflammation.
• Antinuclear antibody: A test for antinuclear antibodies, which are often found in those with autoimmune disorders, including JRA.
• Cyclic citrullinated peptide (CCP): Although CCP is not routinely tested for in children, in some cases doctors may carry out tests for the presence of CCP antibodies which can then sometimes indicate JRA.

The results of these tests can help doctors in making a diagnosis.

Visits to an ophthalmologist (a doctor specializing in eye care) are necessary to check for eye inflammation, both in the process of diagnosing JRA and to monitor disease progression. Left untreated, prolonged eye inflammation may lead to vision loss.

Imaging scans – such as X-rays or magnetic resonance imaging (MRI) – can be used to help doctors identify any fractures, bone erosion, infections and any other damage which may help in their diagnosis.

The aim of JRA treatment is to minimize pain, retain one’s range of motion and muscle function, facilitate normal growth and development, as well as to prevent serious complications. To achieve this, a long-term treatment plan involving a combination of drugs and physical therapy will typically be necessary. 9

In very rare cases, surgery may also be called for to help repair joint or bone damage caused by the condition.

A range of medications are available to help treat JRA. Due to the severity of the side-effects that may be caused by many of these medications, doctors will usually be cautious about prescribing large doses, especially in the early stages of the condition. As the condition progresses and if symptoms become more severe, larger doses may be called for.

Some of the most commonly prescribed medications for treating JRA include: 10 11 12 13

Nonsteroidal anti-inflammatory drugs (NSAIDs): In most cases, NSAIDs will be used in the initial stages of treatment to help relieve pain and ease inflammation. This type of medication also has few side-effects. Some NSAIDs are available over the counter (ibuprofen, for example), while others need to be prescribed by a doctor. Although NSAIDs can help to relieve certain symptoms, they do not affect the overall prognosis of JRA.

Corticosteroids: Used to treat inflammation, pain and stiffness. These can be taken orally or injected directly into the affected area, which is particularly effective as a form of pain relief. They may lead to serious side effects (including weakened bones) if taken over a long period.

Disease-modifying antirheumatic drugs (DMARDs): This type of medication works by altering the way the immune system works. They are generally prefered by doctors treating JRA because of their lack of side effects and general efficacy. Methotrexate is the most commonly administered type of DMARD.

Biological response modifiers: Generally reserved for the treatment of those who have not responded to DMARDs, this type of medication works by targeting the immune system and modifying the molecules causing the inflammation. They are usually administered through injection or infusion. Commonly prescribed drugs in this category are Etanercept or Adalimumab.

Although movement may become painful for people with JRA, it is important to try and maintain an active, healthy lifestyle. This has the effect of keeping muscles and joints strong and flexible, and may reduce inflammation. A physical therapist will typically be recommended to help those with the condition to design an appropriate exercise plan which suits their individual needs.

Although there are no specialist guidelines regarding diet for those with JRA, it is generally recommended that individuals eat a healthy and balanced diet. It may also be helpful to consume the following types of food, each known to contain anti-inflammatory and antioxidant properties: 14

• Oily fish high in Omega-3 (such as salmon, tuna and mackerel)
• Fruits and vegetables (especially strawberries, blueberries and cherries)
• Citrus fruits (high in vitamin C)
• Nuts
• Beans

Surgery is rare in the treatment of JRA, as medications and physical therapy are generally sufficient. However, in a small number of cases – for example, if the condition is affecting the curvature of the spine – surgery may be performed. The need for surgery will be assessed on a case-by-case basis by doctors. 15

• Juvenile idiopathic arthritis

Q: What happens to people with juvenile rheumatoid arthritis after the age of 16?A: It may seem logical to think of JRA as simply a form of rheumatoid arthritis (RA) experienced by people under the age of 16. In reality, JRA is a distinct condition with its own characteristics. For this reason, it not usual for JRA to simply become or graduate to rheumatoid arthritis in people over the age of 16. Instead, it is better to describe people in this situation as adults with a history of juvenile rheumatoid arthritis.

In rare cases, however, children diagnosed with JRA may actually have RA. If this is identified, it will then be accurate to describe the condition as RA. In other rare cases, the symptoms of JRA may fade as a child enters adulthood. This is typically the case in those who are diagnosed with JRA at a very young age and experience a mild form of the condition. 16

Q: What is the life expectancy for people with juvenile rheumatoid arthritis?A: It is difficult to give an accurate prognosis in cases of JRA. The condition is typically experienced throughout one’s life, but with proper treatment and management its symptoms can be effectively controlled. However, average life expectancies for people with JRA are generally shorter than those for people without the condition. The main reason for this is not the condition itself but the complications which can arise from the condition. 17