Long-term outcome in patients with juvenile idiopathic arthritis. (Arthritis Rheum, 2002) (3).
In this descriptive case series of 2 populations of children with juvenile idiopathic arthritis (JIA) seen in Germany between 1978 and 1988, fewer than 10% of subjects were severely disabled or handicapped after more than 15 years of disease duration. Of the 260 eligible subjects (all older than 15 years of age by December 1999), 215 were studied by survey and by direct clinical examination. Assessment tools included the Health Assessment Questionnaire (HAQ), global assessments (of wellbeing, level of function and pain, etc), joint counts (including a 42-joint count), and the Steinbrocker functional classification. The American College of Rheumatology (ACR) criteria for clinical remission of rheumatoid arthritis (RA) were used to classify patients into complete remission categories (e.g., no active disease and no antirheumatic therapy) or partial remission (no active disease on antirheumatic therapy).
Median duration of symptoms was 4 months at initial evaluation and the median followup period was 16.5 years. Approximately 40% of the group had oligoarthritis, 14% had systemic-onset disease, and 33% had psoriatic, enthesitis-related, or other forms of arthritis. Median duration of disease was 4 months at initial evaluation, and the median duration of followup was 16.5 years. Approximately one-third of the oligoarticular-onset cases had polyarticular extension.
Approximately 15% of the patients developed uveitis after a median disease duration of 4 years. Half of the patients developed sequelae including reduced visual acuity and 2 patients (1% of total cohort) lost their eyesight. At last followup, 4 of these patients were still receiving treatment for uveitis. No deaths were reported in this cohort.
Three patients achieved a final height below the third percentile (2 patients with systemic onset arthritis, and 1 with polyarticular arthritis). Approximately 25% of the total cohort had limb length discrepancies averaging one centimeter, and 33% of these required shoe lifts. Micrognathia occurred in 22% of patients with polyarticular disease, in 13% of patients with systemic-onset disease, and in 8% of those with oligoarticular-onset disease.
Approximately half (45%) of the children had a surgical procedure sometime during the course of their disease, the majority of which were synovectomies. Only 5 patients underwent joint replacement surgery, after an average disease duration of 13 years.
At the last followup visit, 40% of the cohort had complete remission, and 5% had partial remission. Children with oligoarthritis had the best chance of complete or partial remission (59%), followed by those with systemic-onset disease (54%) and polyarthritis (41%). Approximately 40% of the cohort had swollen or tender joints at the final followup visit, and almost 60% had joints with limited ROM. Approximately 50% of the patients reported joint pain, and ∼33% reported morning stiffness at final followup visit. About 20% reported using a nonsteroidal antiinflammatory drug and about 25% reported taking a disease-modifying antirheumatic drug (DMARD) at their final visit.
Sixty-one percent of the cohort reported a HAQ score of zero, and 6.5% reported a HAQ score ≥1.0, which correlated well with the Steinbrocker functional classification and disease activity, with trends towards correlation with disease duration and polyarticular disease. Twenty-one patients were classified as Steinbrocker functional class III and 1 patient classified as class IV at last followup visit. There were no significant differences in educational level and employment/vocational status when compared with age-matched controls.
The data from this study can be applied to discussions with patients and families of children with a new diagnosis of JIA. Because JIA includes enthesitis-associated arthropathies, some of the relative percentages would be different for children with JRA. In this long-term study, meaningful clinical and functional outcomes were measured. Approximately 10% were classified as either Steinbrocker functional class III or IV, but no deaths occurred. Overall long-term outcomes of children with JIA from these 2 cohorts are generally favorable.
The early pattern of joint involvement predicts disease progression in children with oligoarticular (pauciarticular) juvenile rheumatoid arthritis. (Arthritis Rheum, 2002) (4).
In this descriptive case series of 205 children with pauciarticular JRA from 2 North American Centers, followed for at least 5 years, ∼40% had extension to >4 joints, and ∼50% of these patients had ≥10 joints involved. The medical records of 365 children diagnosed with oligoarticular JRA from 1965–1994 were reviewed. Two-hundred and five of these children met the ACR criteria for oligoarticular onset JRA, were followed up for at least 5 years, were not rheumatoid factor positive, and did not meet criteria for psoriatic or other seronegative spondylarthropathies during the first 6 months of their disease.
The median age at diagnosis was 4.9 years, ∼80% were female, and the median followup was 10.8 years. Approximately 40% had >4 joints involved after 6 months, and 18% had >10 joints involved. Nine percent of this cohort went on to develop a more clear-cut spondylarthropathy. The presence of symmetric joint involvement, ankle or wrist involvement, and an elevated erythrocyte sedimentation rate (ESR) significantly increased the likelihood of extension to at least 10 joints. These findings were also associated with a statistically significant increased likelihood of erosions, use of DMARDs, persistent disease activity, and other negative outcomes.
An important observation from this study is that almost 20% of children with rheumatoid factor negative JRA, who presented with oligoarticular disease, extended to at least 10 affected joints. Risk factors for this extension included wrist or ankle involvement, symmetric disease, and an elevated ESR. In addition, this group was more likely to have other adverse clinical and radiographic outcomes. Children who present with oligoarticular JRA, with these risk factors should be monitored closely for extension into polyarticular disease.