Medical Overview

JRA, also referred to as juvenile idiopathic arthritis, is the most frequent connective tissue disease of childhood (Olson 2003). The etiology of JRA is unknown but may include a combination of factors such as environment (e.g., infection, trauma, stress), autoimmunity, and immunogenetics (Drotar 2006).

Diagnostic criteria for JRA include an age of onset of younger than 16 years of age, arthritis of one or more joints, and a duration of disease greater than or equal to 6 weeks; other conditions that present with arthritis in childhood must be excluded (Arnett et al. 1988).

JRA is composed of three main subtypes that all share the feature of chronic joint inflammation. These distinct subtypes—pauciarticular (oligarticu-lar) disease, polyarticular disease, and systemic disease—vary in their onsets, symptoms, treatments, and outcomes (see Table 24-1). Diagnosis of each subtype is based on the number of joints involved during the first 6 months of illness. Specifically, pauciarticular disease is associated with four or fewer joints, polyarticular is associated with five joints or more, and systemic disease is associated with multiple systems of the body and rash or fever (Drotar 2006).

Pauciarticular disease is the most common form of JRA, affecting more than one-half of all children with JRA. Data indicate that this subtype is more common in girls and in children younger than 8 years. Areas of the body most commonly affected by pauciarticular disease include the larger joints such

This chapter was supported in part by a grant award from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (1 P60 AR049459-01).

Table 24-1. Three major subtypes of juvenile rheumatoid arthritis (JRA)

Pauciarticular (oligarticular) disease

Polyarticular disease

Systemic disease


Typically in children

Any age

Any age, but most frequently

in early childhood (younger

than 8 years of age)


More than 50% of all

30%-40% of all cases of

10%-20% of all cases of JRA

cases of JRA



More common in

More common in females

Equal frequency in males and



Joint involvement

Four or fewer joints

Five joints or more

Multiple systems of the body

Affected areas

Larger joints—knees,

Smaller joints—hands,

Multiple systems—high

shoulders, elbows,

feet. Occurs in

cyclic fevers, joint

wrists, ankles

symmetrical fashion

inflammation, skin rashes,

internal organ inflammation


Excellent except for

Guarded to moderately

Moderate to poor



as the shoulders, knees, elbows, wrists, and ankles. The prognosis for this subtype is excellent except for eyesight (Cassidy et al. 2005).

Polyarticular disease, which may affect five or more joints in the body, is likely to have an impact on smaller joints, including the hands and the feet, and to occur in a symmetrical fashion. This subtype accounts for about 30%-40% of all cases of JRA and can occur at any age. Similar to pauciarticular disease, the polyarticular disease subtype occurs more in girls than in boys. The prognosis for polyarticular disease is guarded to moderately good (Cassidy et al. 2005).

Systemic disease, also termed Still's disease, is the least common of the subtypes, occurring in about 10%-20% of children with JRA. Multiple systems of the body are affected by systemic disease, including high cyclic fevers, joint inflammation, skin rashes, and inflammation affecting internal organs, including the heart, liver, and spleen. This subtype can begin at any age, although the most frequent onset is during early childhood. In contrast to the other two subtypes, systemic disease occurs with equal frequency in boys and girls. Finally, systemic JRA is considered the most difficult subtype to treat, with up to 25% of children evidencing poor response to their disease, resulting in a moderate to poor prognosis (Cassidy et al. 2005).

JRA frequently results in short- and long-term disability due to joint destruction and other complications (Cassidy et al. 2005) and often extends past adolescence into adulthood (Oen 2002). Some of the more common symptoms experienced by children with JRA include acute and chronic pain, persistent inflammation of multiple joints, decreased mobility, excessive stiffness, and growth retardation (Connelly 2005). These complications often result in frequent visits to the doctor, restrictions of age-appropriate activities, and chronic school absenteeism (Connelly 2005). Damage resulting from joint destruction and other complications most frequently occurs early in the course of the disease. The course of the disease is considered unpredictable, but it most often includes varying periods of flares and remissions (Ward et al. 2008).

Medical management of JRA tends to be aggressive early in the disease and primarily focuses on reducing pain and inflammation, preventing joint deformities and destruction, maintaining complete function in affected joints, and identifying, treating, and preventing any complications (Rapoff et al. 2005). As such, the treatment of JRA is considered ameliorative and not curative. The most common treatment options for JRA include pharmacother-apy, psychosocial support, and physical and occupational therapy (Cassidy et al. 2005). Nonsteroidal anti-inflammatory drugs (NSAIDs; e.g., ibuprofen) are the first line of treatment for JRA because of their efficacy in reducing inflammation, swelling, and pain. Additional pharmacological options include cyclo-oxygenase 2 selective inhibitors, corticoster-oids, disease-modifying antirheumatic drugs (e.g., methotrexate), or slow-acting antirheumatic drugs. Psychosocial support techniques focus on pain relief techniques that assist the affected child in controlling pain caused by JRA. Finally, the goals of physical and occupational therapy are to preserve and improve range of motion, maintain the flexibility of joints, increase muscle strength, and reduce pain.

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