Current and Past Functional History

Physiatrists should obtain a thorough current and past functional history. Some pertinent questions include the following: (a) How long does it take the child to get out of bed? (b) Is the pain worse after sitting or lying a while? (c) Compared to prior visits, what is the child’s activity level? (d) Is the child still able to keep up with peers, or has he or she become more of a reader or video game player? (e) Is the child doing well (academically and socially) at school and can the child carry needed supplies from class to class? (f) Has the child been attending physical and occupational therapies? (g) Is the child performing the exercises in his or her home exercise program?

Social history/level of family support: The following are important questions to ask: (a) Who lives with the child? (b) Does the family encourage activity within limits of pain and does the family include the child in activities? (c) Is the child part of a social group at school and outside of school? (d) Is the child given his or her medications as written and is he or she willing to take them? (e) Are there any environmental factors contributing to noncompliance? (f) Are the child’s treatments covered by insurance?

Review of Systems Related to JIA

Pain and other specific complaints can occur with each subtype of JIA in different patterns and should be reviewed at each visit. Important questions to ask include the following: (a) Is sleep interrupted by pain? (b) Does the child have pain on awakening or later in the day after activities? (c) Which joints are affected by pain? (d) Have any joints been swollen? (e) How much and what type of pain medicine does the child take? (f) Is the child growing and gaining weight appropriately?

Oligoarticular JIA generally causes pain in the large joints. Polyarticular JIA tends to cause pain in the small joints of the hands and feet. The cervical spine and temporomandibular joint can also be involved. Children with systemic JIA may complain of fevers twice daily and evanescent rash possibly with Keener phenomenon (skin lesions along line of trauma). Uveitis is also associated with JIA and the child may have symptoms of a red, painful, photophobic eye that can be insidious in presentation.

PHYSICAL EXAMINATION

JIA presentation may be subtle. The most common presentation is a single swollen knee. Other small and/or large joints may have synovitis that is not always symmetric. Polyarticular JIA usually involves knees, wrists, and ankles. Children may have subcutaneous nodules. Boutonniere and swan neck deformities can also be seen. Range of motion (ROM) and flexibility may be decreased. Axial joints can also be affected. Later in the disease, asymmetric growth can be seen and patients may develop torticollis due to cervical spine involvement, decreased oral opening, and micrognathia due to temporomandibular joint involvement, conductive hearing loss due to middle ear involvement, hoarseness due to cricoarytenoid cartilage involvement, rheumatoid nodules, nail pitting, rash, and gait disturbance. Enthesitis-related JIA may have point tenderness over tendon insertion sites (tibial tuberosity and posterior heel) and mildly decreased ROM (ROM 0). Systemic JIA may also have pericarditis or myocarditis, splenomegaly, and lymphadenopathy (2). Children with enthesitis-related JIA might complain of pain, stiffness, and decreased mobility in the lower back, as well as red, painful, photophobic eyes (uveitis). Children with psoriatictype JIA demonstrate involvement of the hands and feet such as protruding sausage digits and nail bed changes.

Laboratory studies: There may be evidence of leukocytosis, thrombocytosis, elevated liver enzymes, and increased acutephase reactants. The clinician should not miss the potentially life-threatening macrophage activation syndrome (MAS) that is associated with systemic JIA. A common sign of MAS is a low erythrocyte sedimentation rate (ESR) (in contrast with a high sedimentation rate in systemic JIA exacerbation).

LONG-TERM OUTCOMES OF JIA

Systemic, rheumatoid factor (RF)-negative and -positive polyarthritis have the highest incidence of advanced joint damage and prospects for long-term disability. Radiologic joint damage occurs over time even in the absence of clinical symptoms. A prolonged active disease course leads to increased joint damage (3). Extraarticular damage (eye damage, growth retardation, pubertal delay), subcutaneous atrophy due to intraarticular glucocorticoids, leg-length discrepancies, avascular necrosis, striae rubrae, and abnormal vertebral curve occur often in systemic and enthesitis-related JIA. Pain is common early in the disease course but diminishes as patients complete puberty. Pain frequently keeps patients awake at night and causes daytime fatigue, which affects school, work, and hobbies (4).

IMPAIRMENTS, ACTIVITY LIMITATIONS, AND PARTICIPATION RESTRICTIONS ASSOCIATED WITH JIA

PSYCHOSOCIAL, VOCATIONAL, AND EDUCATIONAL ASPECTS OF JIA

Most kids with JIA can expect to lead normal lives (7). However, at some point in the course of disease, JIA does affect relationships between family members as well as intimate relationships. Many patients express fear of becoming pregnant and having children (4). JIA can cause patients to feel different from others, and negatively affect their self-esteem and body image. Good friendships and a sense of belonging to a social group are essential (4). Pain is a major factor during childhood and adolescence of patients with JIA, which can lead to absenteeism and decreased participation in activities. Patients with JIA have more anxiety and depression than healthy peers, which correlate with disability and active disease (8). Recent studies have reported that improvements in medications have been associated with decreased pain. However, patients often complain of the complicated and chronic medication regimen, the side effects, and the perceived lack of education regarding the medications. Individuals with JIA have expressed concern that JIA affected their relationships with siblings and worry that their parents do not receive enough outside support. Patients are also afraid of the future and how the disease will evolve (4).

A recent systematic review of qualitative studies revealed very invaluable insights into how children with JIA are coping and dealing with their chronic disease. One important insight was that children do not feel normal when they compare themselves to their peers. Children with JIA have to ask for help with certain tasks and feel that they are treated differently due to their disease if others around them are aware of it. They also feel that no one quite understands their disease or can even empathize with the unremitting pain that they suffer from on a regular basis. Physiatrists are adept at treating chronic pain and chronic diseases, which provides them ample experience that can be utilized in relating to children with JIA. Therefore, they can gain rapport with patients and their families by offering compassion and understanding of what the child with JIA has to endure (9).

Quality of life can be hindered in children with JIA. It was found that children with JIA desire to be active participants in their care and feel that health care professionals do not adequately explain the JIA disease process or even relay the reasoning behind why certain treatments were selected. Children with JIA are interested in being continually educated about all aspects of JIA including activity restrictions and therapy programs. Patients with JIA in general do not fully understand the benefits of exercise; therefore, it is vital to relay to patients and families that a therapy program as an outpatient or one instituted at home is necessary to alleviate joint stiffening and keeping joints at their optimum functioning. Physiatrists have the unique ability to work collaboratively with patients in designing an individualized strengthening and stretching therapy regimen that will lead to less pain, participation in more activities, and provide them with a sense that they have some control over certain aspects of their disease and bodies. It will also foster confidence and establish a strong patient and physician dynamic. Physiatrists and therapists can integrate child-friendly activities with a wide assortment of physical games into the patients’ therapy programs, so that patients are more likely to be compliant with their physical and occupational therapy plans (9).

It is important to be aware that JIA can decrease patients’ participation in school activities, hobbies, and jobs (4). Weiss et al. found that 30% of patients with JIA do not graduate from high school, and 30% are unemployed (8). Federal and state programs may provide assistance to children with JIA with school accommodations or services (Federal Act 504). The Juvenile Arthritis Alliance has resources related to education and vocation (7).

INJURIES ASSOCIATED WITH JIA

Patients with JIA have a higher fracture risk due to lower bone mass. Factors such as disease process, medication side effects, and physical inactivity combine to disrupt bone development and homeostasis. Long bones and vertebra are most at risk (10). Joint replacement surgeries may be needed in up to 72% of JIA patients, primarily those with the oligoarticular, polyarticular, and systemic subtypes. RF-positive patients have the greatest number of joint surgeries (8).

REHABILITATION OF THE PATIENT WITH JIA

JIA involves a cycle of symptoms that decrease physical activity, which in turn worsens the symptoms. Localized arthritis, decreased joint use, and glucocorticoid use all contribute to muscle atrophy. Children with JIA have reduced strength when compared to peers (11). Physical and occupational therapy can help maintain or increase joint ROM, reduce pain, and increase function strength and endurance. Gentle ROM, cold packs, heat, and appropriate rest during flares are key. ROM with passive extension more than flexion two to three times a day helps preserve joint ROM. Gentle relative rest helps decrease fatigue; resting in prone position helps decrease contractures from forming at the hip and knees.

Exercise and Sports

Regular exercise ranging from low to high intensity has been shown to improve joint ROM, increase muscle strength, improve clinical symptoms, and improve health-related quality of life (11). In fact, studies show that exercise is a vital part of treatment in children with JIA. Inactivity decreases both aerobic and anaerobic capacities, and in combination with the disease can lead to deconditioning and further disability (6). Tai chi and yoga are recommended (6). Sports are not contraindicated, and participation in sports will not exacerbate the disease. Children with JIA can participate in any sport of interest, including impact activities and competitive contact sports, if their disease is well controlled and they have adequate physical capacity. Children with neck arthritis should have radiographic screening of atlantoaxial joints, with abnormal films needing further evaluation. Participation should be limited based on pain. As in the general population, children with JIA should wear appropriate protective equipment, including mouth guards and eye protection (12). Exercises in water showed benefits similar to those of land-based programs (13). It is very important to note that multiple studies have reported no adverse events related to exercise training (11).

MEDICAL KNOWLEDGE

GOALS

Demonstrate knowledge of established and evolving biomedical, clinical epidemiological, and sociobehavioral sciences pertaining to JIA, as well as the application of this knowledge to guide holistic patient care.

OBJECTIVES

JIA is the most common rheumatic disease of childhood; 50% to 75% of patients seen in pediatric rheumatology referral clinics are due to JIA (1); 1 in 1,000 children is affected with JIA. Epidemiologic studies report the incidence of JIA from 1 to 22 per 100,000 with a prevalence of 8 to 150 per 100,000. European descent appeared to be an important predisposing factor for oligoarticular JIA; in addition, psoriatic JIA is also more common in patients of European descent. Black and Native American patients are more likely to have RF-positive polyarthritis. In general, girls are more often affected than boys. Females are more affected with oligoarticular and polyarticular JIA, and males are more affected with enthesitis-related JIA. Systemic subtype is relatively equal between the sexes. Age ranges of JIA development are median 5 to 8 years of onset, earliest with oligoarthritis and latest with seropositive polyarthritis (14).

JIA involves infiltration of the synovium by lymphocytes and macrophages with production of fibroblasts and macrophage-like synoviocytes. The anatomy of a growing child’s joint differs from that of adults. Children have thicker cartilage than adults due to ossification still being in progress; in addition, cartilage in children is able to renew itself better than in adults. Patients with JIA have been shown to improve radiographically with or even without treatment. Joint space narrowing is the most common form of radiographic damage in JIA (15).

The cause of JIA continues to remain inexact. JIA is hypothesized to be due to multifaceted genetic traits involved in immunity and inflammation that predispose one to develop JIA. When these genes are triggered by environmental factors such as stress, joint trauma, infection, or imbalanced hormones, then disease arises (8).

Particular human leukocyte antigen (HLA) class I and class II alleles have been found to be linked to an increased risk of JIA. Inflammation also is involved. Serum levels of circulating immune complexes have been found in JIA, as have antinuclear antibodies (ANAs), C-reactive protein (CRP), and RF. Synovial fluid of JIA patients has revealed T cells and increased amounts of interleukins (15).

JIA, previously called juvenile rheumatoid arthritis, refers to a complex collection of rheumatologic disorders characterized by chronic arthritis and prolonged synovial inflammation that causes joint deterioration, thereby leading to diminished function and worsening of quality of life (15).

JIA is characterized by (1):

Patients have a similar set of complaints such as joint heat, joint pain, morning stiffness that improves throughout the day, “gelling phenomenon,” decreased ROM of a joint, and difficulty performing activities of daily living (ADLs) (1). The International League of Associations for Rheumatology (ILAR) has set specific criteria for classification of JIA subtypes. There are 7 subtypes of juvenile arthritis: oligoarticular, polyarticular RF-positive, polyarticular RF-negative, systemic, enthesitis related, psoriatic, and “other” (15).

In polyarticular JIA, patients have five or more affected joints within the first 6 months of disease. Patients can have RF-negative and RF-positive diseases, and girls are more affected in general than boys. This subtype has no strong HLA association. The seronegative patients develop polyarticular JIA in early childhood. The seropositive polyarticular JIA patients are usually girls who develop severe erosive disease in late childhood and adolescence in symmetric pattern in small joints (14).

In systemic JIA, patients develop systemic extraarticular manifestations such as salmon-pink macular rash, 2 weeks of high-spiking twice-daily fever, lymphadenopathy, hepatosplenomegaly, pericardial effusions, and serositis. Patients then usually develop polyarticular joint involvement within 6 months of systemic symptoms. Uveitis is a very rare occurrence (8).

Enthesitis-related arthritis commonly involves the lower limbs, especially the hip. It is characterized by inflammation at the insertion of tendons, ligaments, or joint capsules such as bone iliac crest, posterior and anterior superior iliac spine, femoral greater trochanter, ischial tuberosity, patella, tibial tuberosity, Achilles, and plantar fascia insertion sites. It can also involve the sacroiliac joints (14).

In psoriatic JIA, psoriasis and arthritis may not occur at the same time. Positive laboratory markers include ANA and HLA-B27. Psoriatic arthritic JIA patients have asymmetric arthritis that can affect both large and small joints. If the rash is absent, then the diagnosis can be made if the patient has a family history of psoriasis in a first-degree relative, dactylitis, and nail pitting. These patients are at risk for iritis and need frequent ocular evaluations (14).