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Introduction

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

Although many children with inflammatory arthritis enter long-term remission before entering adulthood, a substantial proportion exhibit persistent disease and require ongoing rheumatologic care beyond the pediatric years. These patients have medical needs that differ, occasionally substantially, from those of patients with adult-onset rheumatic diseases. This review will summarize outcomes data in juvenile-onset arthritis and discuss the medical management of these patients as adults, including the transition from pediatric to adult care.

Classification of juvenile arthritis

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

The 1977 American College of Rheumatology criteria described 3 principal subsets of juvenile rheumatoid arthritis (JRA): pauciarticular (pauci-onset JRA) with <5 joints involved in the first 6 months of disease; polyarticular (poly-onset JRA) with ≥5 joints affected in this period; and systemic, where the onset of disease is marked by fevers, rashes, and other systemic symptoms (1). Pauci-onset JRA has 2 clinical subpopulations, a milder form in which no more than 4 joints are ever involved and an extended form in which the disease progresses to affect ≥5 joints over time. Poly-onset JRA also has 2 subtypes differentiated by the presence of rheumatoid factor (RF), with the RF-positive subset (10% or less of all JRA) being essentially identical to adult seropositive rheumatoid arthritis. Dissatisfaction with limitations in the JRA classification as well as its European counterpart, juvenile chronic arthritis, has led to a recategorization of the primary childhood arthritides under a more recent nomenclature, juvenile idiopathic arthritis (2). Because the bulk of outcomes research has used the JRA classification, and this terminology remains most familiar to many rheumatologists, we will use this classification system for the purposes of the current review (3).

Outcome of juvenile rheumatoid arthritis

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

Studies on the outcome of childhood arthritis fall into 2 groups. Most commonly, patients are identified on the basis of pediatric clinic records and an attempt is made to assess how they have fared with time (Table 1) (4–24). Such studies are subject to varying degrees of ascertainment bias in the accumulation of cohorts and the collection of long-term data, potentially skewing results toward patients having prolonged contact with the health care system. However, they provide the only available insight into the (treated) natural history of JRA. A second type of study examines JRA patients recruited from adult rheumatology clinics, selecting overtly for severity but best reflecting the population encountered by the rheumatologist who treats the adult patient (25–32). The interpretation of all such studies is complicated by the use of divergent classification criteria and outcome measures, incomplete reporting of data by disease subtype, and advances in management that have made disease outcome a moving target.

Table 1. Outcomes studies in juvenile rheumatoid arthritis (JRA)*
Study yearAuthor (ref)ClassificationNFollowup, yearsPauciarticular n (% remission)Polyarticular n (% remission)Systemic n (% remission)
  • *

    JCA = juvenile chronic arthritis; JIA = juvenile idiopathic arthritis. Data represent an extrapolation into the JRA classification (see reference 3 for review). Table includes all published studies providing at least 7 years of followup and sufficient detail to enable differentiation of outcomes by disease subclass. In the case of multiple studies by the same author, study with the longest followup was selected. The definition of remission varied among studies and may include some patients in complete control through ongoing treatment.

  • Outcomes data provided according to disease course rather than onset type.

1976Calabro (5)JRA1001532 (69)48 (60)20 (75)
1977Hanson (6)JRA1231028 (29)27 (56)60 (42)
1983Svantesson (9)JRA181018 (50)
1984Rennebohm (10)JRA11514.751 (78)42 (50)22 (73)
1987Pedersen (24)JRA931062 (65)25 (60)6 (60)
1987Ansell (4)JCA1561087 (67)39 (48)30 (57)
1995Andersson Gare (12)JCA1017.140 (55)58 (48)3 (66)
1998Flato (17)JRA531032 (72)17 (65)4 (0)
2000Zak (18)JCA652643 (65)17 (53)5 (80)
2002Minden (21)JIA14516.585 (54)30 (27)30 (47)
2003Fantini (23)JCA61610420 (36)108 (18)88 (33)
2002Oen (22)JRA38710220 (47)120 (17)47 (37)

Despite these difficulties, a relatively clear picture emerges: pauci-onset JRA that remains limited to <5 joints remits in approximately 50–75% of patients, while the other subforms tend to persist into adulthood, positive RF poly-onset JRA doing so almost uniformly (Table 2). Although many patients enter long-term remission, no study has suggested any role for puberty in this improvement, i.e., patients may outlast arthritis but they do not “outgrow” it in the sense of discarding it with other elements of childhood physiology (11).

Table 2. Persistent active disease in juvenile rheumatoid arthritis (JRA)*
TypeNActive disease (%)
  • *

    Data for subgroup analysis were available only from a limited number of studies (pauciarticular-onset refs. 5, 17, 18, 21, 23 and polyarticular-onset refs. 10, 21–23, 104).

  • At 7–26-year followup.

  • Data reflect one large study (ref. 23) with 43% remission on mean 10-year followup, and 4 smaller studies (refs. 5, 17, 18, 21) with ≥10 year per patient followup and 73–84% remission rates, likely reflecting the true long-term outcome of persistent pauciarticular JRA.

Pauciarticular-onset JRA (all)1,10051
 Remains limited to <5 joints for entire course44947
 Extension to ≥5 joints16380
Polyarticular-onset JRA (all)52963
 Rheumatoid factor-negative25368
 Rheumatoid factor-positive8693
Systemic onset JRA33354
All JRA1,96955

The outcome of JRA depends on disease subtype and effectiveness of antiinflammatory therapy. Pauci-onset JRA, the most common subtype, generally responds well to treatment and has an excellent prognosis. Major persistent morbidities are leg length discrepancy from overgrowth or undergrowth at the physes surrounding the inflamed knee, and ocular injury from uveitis (33). By contrast, patients with JRA involving ≥5 joints (including JRA that begins as pauci-onset JRA but extends to >4 joints) have a more guarded prognosis. Therefore, among patients with polyarticular or systemic disease for over 25 years and still followed in an adult rheumatology practice, >30% exhibited limitations in self care (Steinbrocker criteria stage III/IV) and >50% had undergone at least 1 joint replacement surgery (28). Although outcomes have improved, pediatric rheumatology patients on the verge of transfer to an adult rheumatology practice still exhibit substantial functional disability (34). While premature death is rare, mortality in patients with JRA exceeds that expected for age by severalfold, and this elevated risk extends to adults with a history of JRA. Causes of death have included sepsis, complications of therapy, amyloidosis, and associated autoimmune conditions (35, 36).

In addition to persistent active inflammation, adult patients with JRA face a number of medical problems relatively specific to this population (Table 3). We will review these issues before discussing an approach to the transitioning patient.

Table 3. Medical problems in adults with juvenile rheumatoid arthritis
Musculoskeletal
 Active inflammatory disease
 Chronic orthopedic deformities
  Inflammatory joint injury
  Growth-related deformities
  Cervical spine disease
  Joint prostheses
Nonarticular complications
 Eye disease
 Osteoporosis
 Amyloidosis
 Reproductive complications
 Cardiovascular risk (unknown)
 Long-term medication toxicity (unknown)
 Associated immunodeficiency and autoimmunity
Psychosocial consequences
 Social dysfunction
 Vocational failure
 Anxiety/depression

Musculoskeletal management of JRA in adulthood

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

Treatment of ongoing synovitis.

Antiinflammatory management in the adult patient with active JRA has not been studied specifically. Typical therapies for children with JRA are listed in Table 4 (37). These treatments overlap completely with those in customary use in adult rheumatology, although dosing regimens differ based on age-dependent pharmacokinetics. By the time JRA patients reach the adult clinic, however, doses and monitoring schedules used for adult rheumatoid patients are appropriate. One possible exception is systemic-onset JRA. In childhood systemic onset JRA, gold and sulfasalazine are relatively contraindicated because of an association with life-threatening macrophage activation syndrome (38). As in all patients of reproductive age, avoidance of sexually transmitted disease and unintended pregnancy is critical, the latter particularly because of possible exposure to known teratogens such as methotrexate and leflunomide.

Table 4. Antiinflammatory therapy for JRA in children*
Onset typeTherapy
  • *

    JRA = juvenile rheumatoid arthritis; NSAIDs = nonsteroidal antiinflammatory drugs; CS = corticosteroids; SSZ = sulfasalazine; MTX = methotrexate; HCQ = hydroxychloroquine; TNF = tumor necrosis factor; IV = intravenous. Clinical trials support is available only for a subset of these treatments; see reference 37 for review.

  • Therapeutic choices are determined by disease course (pauciarticular versus polyarticular) rather than onset type.

PauciarticularNSAIDs, intraarticular CS, SSZ, MTX
PolyarticularNSAIDs, intraarticular CS, oral CS, HCQ, SSZ, MTX, leflunomide, anti-TNF
SystemicNSAIDs, intraarticular CS, oral/IV CS, MTX, leflunomide, anti-TNF, thalidomide, anakinra

The management of the adult JRA patient is frequently complicated by a relative difficulty of assessing disease activity. Inflammation in the immature joint results not only in cartilage loss and bone erosions but also in regional hypo- or hyperplasia, discussed further below. Joint pain of purely biomechanical origin is therefore common despite clinical remission. Morning stiffness is an uncertain guide to distinguish synovitis from mechanical joint pain, and inflammatory markers such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be unimpressive even in patients with active disease (18, 39). In one well-documented series, the ESR was elevated (>10 mm/hour) in less than 50% of adult JRA patients judged clinically to have active disease, while the CRP level was within the normal range in over 70% (12). Therefore, in the antiinflammatory management of the adult with JRA, the rheumatologist is forced to rely on clinical findings and judgment, just as is the case with the adult with long-standing rheumatoid arthritis. Gadolinium-enhanced magnetic resonance imaging and, where available, musculoskeletal ultrasound are potentially useful aids, although experience in this context is limited.

Orthopedic consequences of JRA.

Similar to adult rheumatoid disease, uncontrolled JRA can result in chronic joint injury. However, because inflammation in JRA occurs in the growing skeleton, patients commonly develop orthopedic complications not seen in adult disease. The classic example is leg length discrepancy. The majority of linear growth of the lower extremity occurs at the physes surrounding the knee. In affected children younger than the age of 9, accelerated growth results in modest (≤3 cm) overgrowth of the affected limb. In older children, by contrast, premature closure of the growth plate can result in limb shortening of ≥5 cm (40). Similar changes can occur in the arms, small joints of the hands and feet, as well as at the hip and ankle, with hypoplasia predominating over hyperplasia. Therefore the adult with severe, chronic JRA may have shortened upper extremities, hypoplastic wrists, brachydactyly, small femoral heads and poorly formed acetabuli, and deformed ankles and feet (41, 42). These changes can result in ongoing pain, disfigurement, and particular challenges for the orthopedic surgeon if joint replacement is required.

Two regions of the skeleton exhibit hallmark growth abnormalities. The temporomandibular joint (TMJ) is involved in 40% of patients with JRA, especially in those with poly-onset and systemic disease (43–45). TMJ inflammation can manifest as limited or painful jaw opening, but in the majority of cases the disease is symptomatically silent. Because substantial mandibular growth occurs in the subchondral region of the articulating condyle, inflammation at the TMJ (even if clinically silent) threatens mandibular growth and can lead to micrognathia (5, 43, 46, 47). Risk factors for micrognathia include poly-onset and systemic disease, onset of disease prior to age 4, and prolonged inflammatory activity, although even a brief period of inflammation at a critical developmental time can have long-term consequences for jaw size (7, 44).

A second region of special orthopedic interest in JRA is the cervical spine. Similar to adult rheumatoid arthritis, JRA may affect the atlantoaxial joint, requiring management akin to that appropriate for adults (48). Unlike RA, poly-onset and systemic JRA commonly involve other cervical vertebrae as well, characteristically with fusion of the posterior elements, most typically between C2 and C3 (Figure 1). Such fusion may occur even into early adulthood. Depending on the age of the patient, this process may restrict growth of the vertebrae and disk spaces and result in a shortened neck. Aside from reduced mobility and accompanying pain, the fused vertebral segment provides an exaggerated moment arm and so poses a risk for cervical fracture and pseudoarthrosis. Fortunately, neurologic sequelae remain relatively uncommon, and most patients do not require surgical management (49, 50). As in adult RA, the potential for atlantoaxial disease justifies particular attention to the cervical spine during anesthesia induction and intubation (51). Since cervical spine disease correlates strongly with TMJ involvement, particular care is required in patients with micrognathia (44).

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Figure 1. Cervical spine radiograph of a 24-year-old woman with rheumatoid factor-negative polyarticular juvenile rheumatoid arthritis (disease onset at age 5). Note the fusion of C2 through C4. Prior films taken at age 18 were normal.

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Total joint replacement in the young adult.

Orthopedic surgery in adults with severe chronic JRA may be especially challenging because of contractures, osteopenia, and abnormal bone morphology, such that custom prosthetics are frequently required (51). Total joint replacement in such patients provides excellent relief from pain and impaired mobility but is complicated by the limited lifespan of available prostheses, especially with the heavier use to which young individuals may expose these joints. For hip prostheses, the 15-year survival rate without revision remains in the range of 70–83%, which is somewhat lower than reported for older adults. Acetabular loosening is the primary reason for failure, with steroid use as a principal risk factor (52–54).

Knee replacements fare somewhat better, with success rates and survival comparable with those in older adults, at least in the relatively short term (51, 55, 56). Shoulder replacement is performed less often, but appears to be relatively successful (57). Given the difficulty attendant to revision arthroplasty, the decision to proceed to joint replacement requires individualized risk-benefit analysis.

Extraarticular complications of JRA.

In addition to persistent disease activity and chronic joint injury, for which the adult rheumatologist is well prepared, patients with JRA are subject to a series of comorbidities particular to this patient population. Attention to these issues is integral to comprehensive care of the adult with JRA.

Eye disease.

Patients with pauci-onset JRA and, to a lesser extent, rheumatoid factor-negative poly-onset JRA are at risk for anterior uveitis, especially if arthritis began before age 7 and/or a test result for antinuclear antibodies (ANA) is positive (58). Unlike uveitis in the spondylarthropathies, JRA uveitis is painless and usually unaccompanied by scleral injection, requiring ophthalmologic examination for diagnosis. The uveitis onset is typically within the first 7 years after presentation, but rarely it may initially occur in early adulthood (59). In JRA patients diagnosed with uveitis in childhood, more than half will have persistent activity as adults, especially if joint disease also remains active (59).

In young children, regular screening is vital for early detection of disease, and current recommendations call for periodic examinations “indefinitely” (60). However, adult patients with uveitis commonly note floaters or other subtle symptoms and are likely to seek medical care before the onset of irreversible damage (61), although this is not invariably the case (K. Kotaniemi, personal communication). In our institutions, we do not routinely refer adult JRA patients without prior uveitis to ophthalmology screening, but would consider continued screening in patients with active arthritis, especially if they are ANA positive, and maintain a low threshold for referral should symptoms arise. By contrast, patients with a history of uveitis receive continued specialist followup.

Despite aggressive management, 20% or more of JRA patients with uveitis will have some degree of ocular injury, including cataracts, glaucoma, and partial loss of vision, although these outcomes may be improving with the use of tumor necrosis factor (TNF) inhibitors (62, 63). Many of these complications will first become evident after age 16 (64). Cataracts independent of uveitis may also occur as a consequence of steroid therapy, typically in patients with systemic onset JRA. Children appear to be more susceptible than adults to this complication (28, 65).

Osteoporosis.

Patients with JRA have an elevated risk of early osteoporosis due to interference with the usual acquisition of bone mass in adolescence. Several studies have addressed the extent of such bone disease in adults with juvenile arthritis (66–70). One representative study of patients with a mean age of 32 years found frank osteoporosis of the hip and lumbar spine (T score ≤−2.5) in more than 7%, while osteopenia (T score between −1 and −2.5) was identified in 46% of patients at the hip and 35% in the spine (66). Particular risk is observed in patients with systemic onset and poly-onset disease, prolonged (>1 year) steroid therapy, greater disability as measured by Steinbrocker criteria, and ongoing active inflammation (66, 68–70). However, osteopenia has been noted even in patients who never received corticosteroids (67, 69).

As with all individuals at risk for osteoporosis, patients with JRA should receive education as to the value of exercise, abstinence from smoking, and the need for adequate calcium and vitamin D. Since thyroid disease and autoimmune gonadal failure may occasionally accompany JRA (discussed below), secondary causes of osteoporosis should be considered in patients with an unusual degree of bone loss.

Bisphosphonate therapy in the younger individual may be useful in selected circumstances, although the long-term consequences of these drugs remain to be determined. This is of particular concern in women of reproductive age, because bisphosphonates elute from the bone in small quantities for decades, cross the placenta, and alter fetal bone growth when administered in supratherapeutic doses to rodents (71). However, in the absence of other effective therapies, bisphosphonate therapy use in premenopausal girls and women is expanding, so far without the appearance of case reports of fetal harm (71, 72). Bone density testing should be obtained if bisphosphonates are being considered. In growth-restricted patients, results of dual-energy x-ray absorptiometry scanning require adjustment for bone size to avoid overestimation of the degree of osteopenia (73).

Amyloidosis.

Amyloidosis of the reactive AA type may occur in JRA as a consequence of ongoing systemic inflammation. In one long-term European series, almost 10% of RF-negative poly-onset JRA patients and over 19% of systemic onset JRA patients still being followed clinically developed amyloidosis, not including other patients who had died of this condition (28). Equivalent prevalence has not been seen in a North American series, although the reasons behind the difference remain unclear; even in Europe the rate of amyloidosis has been dropping (74). Amyloidosis occurs primarily in patients with systemic onset and poly-onset disease, presenting as proteinuria with or without hypertension, splenomegaly, hepatomegaly, and abdominal pain as early as 1 year or as many as 20 years after diagnosis of JRA (75, 76). Renal vein thrombosis may complicate the proteinuria (75). Thyroid deposition of amyloid with visible goiter has also been observed (77). In an earlier series, mortality of patients with JRA and amyloid was >50% at 10 years, due principally to renal failure and infection (75, 78). Diagnosis is by fat pad biopsy. Treatment is aimed at strict control of the underlying inflammatory disease, traditionally with chlorambucil, potentially resulting in the reversal of deposition and clinical improvement (78). With current antiinflammatory therapy, it would be expected that amyloidosis should be rare, and indeed successful treatment of amyloid in JRA with TNF inhibitors has recently been reported (79).

Reproduction.

Although most patients with JRA requiring ongoing care into adulthood are sexually active, ∼50% report either physical or psychological limitations in sexual behavior related to their disease (30, 80). One series reported premature ovarian failure in 3% of adult women with JRA (1.7% before age 30) but none was found in another series of 161 patients (81, 82). Other problems observed included an increased incidence of metrorrhagia, pelvic inflammatory disease, surgery for ovarian cysts, spontaneous fetal loss (20% with JRA versus 10% in controls), and difficulty conceiving (11% versus 2% in controls) (82). In women with hip disease or pelvic growth restriction, caesarean delivery is often required, although fetal outcomes are generally normal (30, 83). Inflammatory disease activity in JRA tends to remit during pregnancy, but may flare in the postpartum period (83, 84). Physical limitations and misperceptions about the heritability of JRA may drive the decision to limit the number of children, and some patients report being counseled by physicians and family members to avoid childbearing altogether (82, 83, 85).

Cardiovascular risk.

Adult rheumatoid arthritis is now increasingly recognized as an established risk factor for premature atherosclerotic cardiovascular disease (86, 87). It has been observed that patients with JRA have elevated triglycerides and low high-density lipoprotein cholesterol levels compared with age-matched controls, although levels of low-density lipoprotein cholesterol were also low (88). One study noted reduced aortic distensibility in patients with JRA, potentially consistent with early atherosclerosis (89). However, these studies have not been repeated and no long-term outcome data are available. Since elevated long-term cardiovascular risk is clearly a possibility in JRA, especially in disease that runs a polyarticular or systemic course, attention to modifiable cardiovascular risk factors is prudent until more definitive studies are available.

Medication toxicity.

While the safety profile of disease-modifying agents in children appears generally favorable, the long-term consequences of decades of use remain unknown. Clinical and liver biopsy data from JRA patients treated with methotrexate therapy are reassuring, but still represent relatively short-term results (90, 91).

Associated autoimmune conditions.

Similar to other autoimmune diseases, JRA carries an association with a variety of aberrant immune states. These include type I diabetes, thyroid disease, celiac disease, gonadal failure, and immunodeficiencies such as IgA deficiency and common variable immunodeficiency (36, 81, 92, 93). In some series, substantial adult mortality has been attributed to these comorbid states (36). Similarly, the consequences of prolonged childhood exposure to immunomodulatory agents on the ultimate development of the immune repertoire remain undetermined.

Psychosocial consequences of JRA

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

As a potentially disfiguring and disabling disease, JRA not surprisingly carries psychosocial consequences. Half of patients requiring ongoing care as adults report that JRA adversely affects their body image and ability to interact socially with others (30). Patients tend to remain single longer and marry later than their siblings. One-quarter report disease-related discrimination in social settings and at the place of employment, a fact that may contribute to the striking increase in unemployment among adults with JRA in most studies (25–40%) despite higher general educational attainment as measured by completion of a postsecondary school degree (22, 29, 32). Nevertheless, educational success remains critical within this patient population, since poor educational achievement exceeds even physical disability as a predictor of vocational failure (29).

Similarly, quality of life has been noted to be generally poorer among patients with JRA followed into adulthood than in matched controls, driven particularly by physical factors (pain, fatigue, limited function) and somewhat less by social and emotional distress (32). The large majority of JRA patients in adult rheumatology clinics will have some degree of pain (17, 31). While frank depression does not appear to be elevated in adult JRA patients compared with the general population, more than 20% experience a period of depression, most commonly in late adolescence and early adulthood, potentially complicating their transfer from pediatric to adult health care providers (31). Elevated levels of anxiety were found in 31% in this series, well in excess of the 18% expected from the normal population, and were concentrated in patients whose JRA began in adolescence rather than early childhood.

Such outcomes data have important therapeutic implications for the caregivers of adults with JRA. As in any chronic illness, psychological comorbidities should be addressed directly, often with the aid of a counselor or occasionally a psychiatrist. Pain should be acknowledged and treated. Vocational assistance and encouragement are important, although the ongoing need for comprehensive health insurance can sometimes drive the desire to remain on the disability rolls. Social isolation and distress may be ameliorated to some degree via counseling or local support groups composed of young adults with arthritis.

The psychosocial effects of JRA may also complicate medical management. Experience at our centers suggests that adult patients with JRA often cope with illness differently than like-aged patients with arthritis of adult onset. The latter encounter illness as an obstacle in the way of well-established life goals, and use all the resources developed through adolescence to manage the disease in such a way as to maximally achieve preexisting career and personal aims. By contrast, patients with severe arthritis through childhood and adolescence, especially dating from times when effective therapy was less available, may exhibit a more passive approach, accepting limits, fatigue, and pain while altering personal goals to accommodate the disease. This is true particularly for patients in their late teens and early twenties; however, by the mid-twenties patients often return with a new focus on disease management. Correspondingly, one of the tasks of the clinician caring for adults with JRA is to encourage patients to take full advantage of treatment options.

Managing the transition from pediatric to adult rheumatology

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

Since many individuals with JRA will continue to manifest disease activity into adulthood, there is a need for the care of this population by adult rheumatologists. The transition from pediatric to adult rheumatology has been the subject of careful attention (34, 94–99). For these patients, many things are happening at once: increasing independence from parental oversight; loss of relationships with pediatrician, pediatric rheumatologist, and other specialty care providers; entry into a new and potentially bewildering adult health care system; and changes (potentially loss) in insurance coverage. More fundamentally, patients leave the pediatric family-centered care model and enter an often far less supportive, patient-centered adult practice, where a high degree of individual responsibility and initiative is assumed. Concrete differences such as the reduced availability of procedural sedation for joint injections in adult rheumatology can be a barrier to successful transfer (34). Experience in other chronic diseases has determined that transition is a time of high risk for “falling between the cracks” with associated adverse outcomes from inadequate and poorly supervised therapy (100).

Difficulties also face the adult rheumatologist when accepting these patients in transfer. Patients with arthritis since childhood are often complex, with ongoing disease activity as well as substantial pain, and potentially require care through multiple specialist services. Medical records transfer is frequently incomplete. Many patients will never have made a physician's appointment unaided, and some may still rely on caregivers for the administration of medication. Together, these factors add up to a substantial workload for the adult rheumatologist, and the physician may have neither time nor training to take it on (34, 99, 101).

There remains, as yet, no standardized way to address this transition period, although intense study is underway (34). Dedicated centers for the followup of patients with JRA have been established in a handful of locations, but remain the exception rather than the rule (98, 102). In one well-described model operative in Vancouver since 1995, patients are introduced to the concept of expanded personal responsibility in the mid-teen years and are then transferred at approximately age 18 to a structured, transition clinic staffed collaboratively by a pediatric rheumatologist, adult rheumatologist, nurse specialist, physical therapist, and social worker. In this setting, patients are encouraged to assume increasing independence in their own care, resulting in ultimate transfer to an adult rheumatology practice at approximately age 22 (98). While this remains a pioneering model, it is far from certain that a similar resource-intensive system could be instituted more generally, especially given the constraints imposed by health care financing in the US.

Indeed, the critical window for intervention may occur well before the earliest age at which transition is typically contemplated. By the age of 18, patients with severe JRA who have been relieved of responsibility for their own care through the well-intentioned actions of parents and physicians may have difficulty when faced abruptly with the need for adult decision-making. Having never been asked “What do you want to be when you grow up?” or expected to do household chores or hold a summer job, these patients are far behind their peers in career-directed thinking (102). In fact, interest in a vocation and gainful employment begins early in adolescence, while receptiveness to counseling and guidance declines through the teenage years (103). As a result, the optimal time for intervention aimed at ultimate vocational success appears to be at approximately 12–14 years of age, a time at which children are developmentally ready to participate in educational activities aimed at securing future career options. The importance of this “window of opportunity” has been borne out by the success of several regional employment readiness programs (102). Delaying this help until the age of transfer to adult care risks a suboptimal outcome. While caretakers for adults with JRA will need to manage such patients, the larger goal of efforts aimed at the transition to adulthood for individuals with JRA should be to equip the next generation of children with the tools they will require to become successful adults despite chronic illness.

Questions for future research

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

While substantial progress has been made in understanding the adult with JRA, many questions remain unanswered. There are no data addressing antiinflammatory therapy in these patients. We do not know the long-term consequences of childhood treatment that extends over decades, highlighting the need for therapeutic registries. Cardiovascular risk remains undetermined, and optimal management of atherogenic risk and bone health in JRA remains unknown. Finally, the manner in which adolescents with JRA are prepared for independence and transitioned to adult centers remains relatively haphazard. While the diversity of local health care structures guarantees that there will be no “one size fits all” solution for this problem, practical measures to smooth the transition need to be defined.

Conclusions

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES

JRA persists into adulthood in a substantial percentage of cases, depending on disease subtype. These patients have medical needs related to, but distinct from those of typical rheumatoid arthritis patients, including treatment of active inflammation, management of typical forms of joint injury, and screening and therapy directed toward extraarticular pathology. While progress in disease management will reduce the morbidity of patients transitioning to adulthood, adult rheumatologists will nevertheless be faced with patients with JRA for years to come.

REFERENCES

  1. Top of page
  2. Introduction
  3. Classification of juvenile arthritis
  4. Outcome of juvenile rheumatoid arthritis
  5. Musculoskeletal management of JRA in adulthood
  6. Psychosocial consequences of JRA
  7. Managing the transition from pediatric to adult rheumatology
  8. Questions for future research
  9. Conclusions
  10. Acknowledgements
  11. REFERENCES