What is Juvenile Rheumatoid Arthritis (JRA)?

Juvenile rheumatoid arthritis, JRA in short, is a form of chronic arthritis in one or more joints persisting for at least 6 weeks and certain other conditions, which can give rise to similar symptoms, are excluded. Disease has many subtypes depending upon the type of onset and defined by clinical symptoms in the first 6 months of disease. Course of the disease is determined by what happens after 6 months of onset.

How common is Arthritis in children?

1/1,000 in a given year. Most of these cases are mild. However, approx. 1/10,000 have severe arthritis. Juvenile rheumatoid arthritis (JRA) is most common, and persists for months or for years at a time.

What are the different types of Juvenile Rheumatoid Arthritis (JRA)?

There are three subtypes of JRA based on symptoms and signs at the onset of the disease. They are called;

  1. Systemic onset
  2. Pauciarticular onset
  3. Polyarticular onset.

Some authors have further subdivided Pauciarticular-onset and polyarticular-onset JRA into  two subsets each as below:

Onset TypeClinical SymptomsSubtypesAssociated Characteristics

SystemicFever, light salmon-colored rash, extra-articular manifestationsNot applicableOrganomegaly and lymphadenopathy sometimes present
PauciarticularFewer than 5 joints involved during the first 6 mo of illnessEarly childhood onsetUsually young females; high incidence of chronic uveitis; antinuclear antibody-positive
Late childhood onsetUsually males older than 8 years; high incidence of sacroiliitis; HLA B27-positive
PolyarticularFive or more joints involved during the first 6 mo of illnessRF-negativeOnset later in childhood
RF-positiveOnset later in childhood; similar to adult RA

How do we treat Juvenile Rheumatoid Arthritis (JRA)?

General Considerations
Goal of the therapy is to minimize or abolish inflammation in joints to improve short-term and long-term outcome and function, also to maintain normal growth and development of the child.

First-line treatment includes non-steroidal anti-inflammatory drugs (NSAIDs). Intra-articular corticosteroid injections have been found safe and effective and it may have beneficialeffects on growth parameters, and can be administered without much pain. Physical and rehabilitation therapy is must for reducing pain and to maintain joint and muscle function.

If pauciarticular onset JRA, where fewer than 5 joints are involved during the first 6 months of illness and if it occurs in young females in early childhood, Â there has been reported a high incidence of chronic uveitis in patients who are positive for antinuclear antibody (ANA). It can be damaging to the eye, hence careful ophthalmologic examination and frequent follow up is essential. Treatment of uveitis with methotrexate and cyclosporin A may be beneficial in this condition.

The adverse reactions associated with the drugs may pose a significantproblem in appropriate treatment. For example, immunosuppressive medications may be associated with increased frequency of infection, complication to vaccine administration and may increased frequency of developing malignancy.Many drugs that are most effective at pain and symptomcontrol, including corticosteroids and NSAIDs, have no effecton erosive disease. Even methotrexate, known to havedisease-modifying activity, may relieve symptoms but may not halt the disease progression in some patients.

When does JRA not behave well and can lead to deformities?

Polyarticular disease at onset andpolyarticular disease course both can lead to significantrisk factors for disability. Other bad risk factors for disability include female gender and the presence of rheumatoidfactor, ANA and HLA B27. Persistent morningstiffness, tenosynovitis, subcutaneous nodules have also been associated with poor outcomes and are associated with early involvementof the small joints of the hands and feet and rapid appearanceof erosions.

Patients with poor prognosticindicators are more difficult to treat and require early and aggressive therapy. One study revealed that the patients with JRA who developed erosions and disability tended to havereceived treatment later than those who did not.

Arthritis is demonic and can strike people in childhood as it strikes them as adults.