The idiopathic musculoskeletal pain syndromes in childhood.

Idiopathic musculoskeletal pain syndromes in children have a variety of manifestations; they can be diffuse or well localized, constant or intermittent, with or without autonomic symptoms and signs, completely incapacitating or not limiting activities, and they can tax the physician's diagnostic skill. A careful history and examination is usually all that is needed to make a diagnosis, although the differential diagnosis is large and might require laboratory and radiographic investigation. Pain and functional assessment help track the progress with therapy. Intense exercise therapy is associated with the best outcome. Psychologic issues should be evaluated to determine if further psychologic intervention is indicated. The medium-term outcome is probably good for most of these children, but the long-term prognosis is unknown. One must be aware that other manifestations of psychologic problems might emerge. By the time these children and their families see the rheumatologist they are desperate and can be frustrating to work with due to their difficulty in accepting any kind of psychologic element to the pain and its associated disability. Nevertheless, it is rewarding to help the children understand and work through their pain so they can resume normal lives.

Kawasaki disease at British Columbia's Children's Hospital.

OBJECTIVES: To describe the clinical features, diagnosis, treatment and outcome of children with Kawasaki disease (KD) treated at a large tertiary care Canadian paediatric hospital and to try to identify correlations between clinical features and the development of coronary artery abnormalities. METHODS: The charts of 176 patients diagnosed with typical, atypical or incomplete KD between 1992 and 2000 at British Columbia's Children's Hospital were reviewed. RESULTS: The male to female ratio was 1.8:1. The median age was 2.5 years (range two months to 14 years), with 8% nine years or older (42% Caucasian, 43% Asian). Cases occurred steadily throughout the year. One hundred two (58%) patients had typical, 18 (10%) patients had atypical and 56 (32%) patients had incomplete KD. The median time from fever onset to first intravenous immunoglobulin (IVIG) was seven days (range two to 49 days), and treatment began within 10 days of fever onset in 134 (76%) patients. All patients received one or more doses of 2 g/kg IVIG. Forty-two (24%) patients received a second dose for nonresponsiveness, of whom 10 (6%) remained nonresponsive. Eight (5%) patients received intravenous methylprednisolone. Forty-eight (27%) patients developed coronary artery abnormalities, with 10 (6%) echogenic abnormalities, 25 (14%) dilatations and 13 (7%) aneurysms (seven giant). No patient with a normal echocardiogram at four to eight weeks developed an abnormality on subsequent study. Fourteen (8%) patients had persistent abnormalities at last follow-up (median 447 days, range 62 to 3272 days): seven dilations and seven aneurysms (six giant). Five of 13 children (39%) who developed aneurysms failed to meet diagnostic criteria for typical KD, and three of those five aneurysms were present at less than one year after diagnosis. Four of eight (50%) patients receiving intravenous methyl-prednisolone for IVIG nonresponsiveness had or developed aneurysms. One patient died. CONCLUSION: Some children diagnosed with KD who fail to meet the diagnostic description develop coronary artery abnormalities. There is a need for a more accurate means of diagnosis to more appropriately use IVIG, an expensive and increasingly scarce resource. The role of corticosteroids remains unclear and a randomized controlled clinical trial to determine their role is needed.

Review for the generalist: The antinuclear antibody test in children - When to use it and what to do with a positive titer.

The antinuclear antibody test (ANA) is a much overused test in pediatrics. The ANA does have a role in serologic testing but it should be a very limited one. It is often ordered as a screening test for rheumatic illnesses in a primary care setting. However, since it has low specificity and sensitivity for most rheumatic and musculoskeletal illnesses in children, it should not be ordered as a screening test for non-specific complaints such as musculoskeletal pain. It should only be used as a diagnostic test for children with probable Systemic Lupus Erythematosus (SLE) or Mixed Connective Tissue Disease, (MCTD) and other possible overlap-like illnesses. Such children should have developed definite signs and symptoms of a disease before the ANA is ordered. This review presents data supporting these conclusions and a review of the ANA literature in adults and children.By limiting ANA testing, primary care providers can avoid needless venipuncture pain, unnecessary referrals, extra medical expenses, and most importantly, significant parental anxieties. It is best not to do the ANA test in most children but if it ordered and is positive in a low titer (<1:640), the results can be ignored if the child is otherwise well and does not have other features of a systemic illness.

Anesthesia for intra-articular corticosteroid injections in juvenile idiopathic arthritis: A survey of pediatric rheumatologists.

OBJECTIVE: To determine the methods of anesthesia currently being used by pediatric rheumatologists when performing intra-articular corticosteroid injections (IACI). STUDY DESIGN: A questionnaire was emailed to all members of the Childhood Arthritis & Rheumatology Research Alliance, a pediatric rheumatology research network in North America. The questionnaire consisted of 11 questions ranging from procedure technique, treatments prescribed for topical anesthesia and oral analgesia, and factors that might affect procedural pain. RESULTS: Seventy-four of 161 physicians (46%) responded to the questionnaire. On average, each physician injected 33 children (median 25, range 1-160) and 43 joints (median 30, range 1-150) yearly. Local anesthesia was used in children on average >/= 8 years (range 2-16 years), with general anesthesia being more frequently used for younger children. All respondents used local anesthesia. The most commonly used methods of local anesthesia were EMLA((R) )cream plus subcutaneous lidocaine (58.8%), ethyl chloride spray only (39.7%), EMLA((R) )cream only (33.8%), subcutaneous lidocaine only (25%), and lidocaine iontophoresis only (11.8%). Buffering of the lidocaine was routinely done only 7.4% of the time. CONCLUSION: Although pediatric rheumatologists in North America perform IACI on a large number of patients each year, a wide variety of methods are used to deliver local anesthesia with no accepted standard of care. More studies are needed to determine the optimal method of local anesthesia delivery to minimize pain associated with IACI.

Is parental coping associated with quality of life in juvenile idiopathic arthritis?

Parents of children with a chronic condition such as juvenile arthritis must cope with greater demands than those living with a healthy child. They must adopt different behaviours in order to lessen the impact on the family structure. Parental coping refers to the parent's specific cognitive and behavioural efforts to reduce or manage a demand on the family system. The aims of this study were: to describe coping in a cohort of parents of children with JIA; to determine whether quality of life is associated with parental coping; to explore whether socio-demographic factors such as child's age, family socioeconomic status and family structure are associated with parental coping. One hundred eighty-two parents caring for a child with JIA completed a postal survey at three times over a one-year period, which included the Juvenile Arthritis Quality of Life Questionnaire (JAQQ), the Coping Health Inventory for Parents (CHIP) and questionnaires describing socio-demographic characteristics. Linear mixed models were employed to analyse the association between the child's quality of life and parental coping. Mean total QoL scores (JAQQ) showed that children experienced difficulty in completing specified activities at most just below 25% of the time and results fall off slightly following the 6 month time point. Mean parental coping scores for the CHIP subscales at baseline were 38.4 +/- 9.0, 33.4 +/- 11.6, 16.5 +/- 6.1, for Maintaining Family Integration (maximum score 57), Maintaining Social Support (maximum score 54) and Understanding the Medical Situation (maximum score 24), respectively. Understanding the Medical Situation was deemed most useful. The child's QoL was associated with parental coping. Parents of children with greater psychosocial dysfunction used more coping behaviours related to Understanding the Medical Situation (beta coefficient, 0.73; 95% CI, 0.01, 1.45). These findings underscore the importance of helping parents of children with JIA better understand their child's medical situation.

Access to pediatric rheumatology subspecialty care in British Columbia, Canada.

OBJECTIVE: Early recognition and treatment of pediatric rheumatic diseases is associated with improved outcome. We documented access to pediatric rheumatology subspecialty care for children in British Columbia (BC), Canada, referred to the pediatric rheumatology clinic at BC Children's Hospital, Vancouver. METHODS: An audit of new patients attending the outpatient clinic from May 2006 to February 2007 was conducted. Parents completed a questionnaire through a guided interview at the initial clinic assessment. Referral dates were obtained from the referral letters. Patients were classified as having rheumatic disease, nonrheumatic disease, or a pain syndrome based on final diagnosis by a pediatric rheumatologist. RESULTS: Data were collected from 124 of 203 eligible new patients. Before pediatric rheumatology assessment, a median of 3 healthcare providers were seen (range 1-11) for a median of 5 visits (range 1-39). Overall, the median time interval from symptom onset to pediatric rheumatology assessment was 268 days (range 13-4989), and the median time interval from symptom onset to referral to pediatric rheumatology was 179 days (range 3-4970). Among patients ultimately diagnosed with rheumatic diseases (n = 53), there was a median of 119 days (range 3-4970) from symptom onset to referral, and 169 days (range 31-4989) from onset to pediatric rheumatology assessment. CONCLUSION: Children and adolescents with rheumatic complaints see multiple care providers for multiple visits before referral to pediatric rheumatology, and there is often a long interval between symptom onset and this referral.

Effects of adherence to treatment on short-term outcomes in children with juvenile idiopathic arthritis.

OBJECTIVE: To determine the impact of adherence to treatment (medication and prescribed exercise) on outcomes in children with juvenile idiopathic arthritis (JIA). METHODS: In this longitudinal study, we studied parents of patients with JIA at the Montreal Children's Hospital and British Columbia Children's Hospital in Vancouver. Adherence was evaluated on a visual analog scale in the Parent Adherence Report Questionnaire. Outcomes of interest were active joint count, pain, child functional score on the Child Health Assessment Questionnaire, quality of life score on the Juvenile Arthritis Quality of Life Questionnaire, and parental global impression of overall well-being. The association between adherence to treatment and subsequent outcomes was evaluated using generalized estimating equations and logistic regression. RESULTS: Mean age and disease duration of our sample of 175 children were 10.2 and 4.1 years, respectively. Moderate adherence to medication was associated with lower active joint count (odds ratio [OR] 0.47, 95% confidence interval [95% CI] 0.22-0.99). Moderate adherence to exercise was associated with better functional score (OR 0.13, 95% CI 0.03-0.54), and lower pain during the last week (OR 0.14, 95% CI 0.04-0.50). Both high and moderate adherence to exercise were associated with parental perception of global improvement. CONCLUSION: Improved outcomes in patients who adhered to treatment underscores the need for clinicians to address adherence issues with their patients. Sustaining adherence, particularly to the more time-consuming treatment of exercise, is a challenge.

Corticosteroid treatment of refractory Kawasaki disease.

OBJECTIVE: To review the indications for corticosteroids in patients with Kawasaki disease (KD) treated by pediatric rheumatologists in Canada and to determine their efficacy on fever in patients with refractory KD. METHODS: All practicing pediatric rheumatologists in Canada identified KD patients treated with corticosteroids and completed a standard data form that included demographics, clinical and laboratory features, imaging studies, and therapeutic interventions, by chart review. RESULTS: Thirty-two patients with KD (14 female; 18 male: mean age 4.6 years) were treated with corticosteroids. Corticosteroids were used in 26 patients (81%) for persistent fever despite treatment with intravenous immunoglobulin (IVIG) (refractory KD), 5 patients (19%) for congestive heart failure, and 1 patient for persistent acute phase symptoms other than fever. The 26 patients with refractory KD are the primary subject of this report. Twenty-two patients (85%) had rapid, sustained resolution of fever after corticosteroids. There were no serious reported adverse effects. Eight patients (31%) treated with corticosteroids developed coronary artery (CA) aneurysms and 9 (35%) developed CA dilatations without aneurysms. Of those who developed CA aneurysm, 4 had aneurysms detected prior to IV methylprednisolone (MP) on echocardiograms performed on days 6-27 (mean day 13) of illness. The remaining 4 patients had CA aneurysm detected after IVMP therapy, on echocardiograms performed on days 13-49 (mean day 23) of illness, 1-25 days (mean 9 days) after IVMP. In patients with one year or more of followup, 46% had resolution of CA abnormalities. CONCLUSION: Corticosteroids are effective in the treatment of fever in most patients with IVIG-refractory KD. A multicenter prospective study is needed to determine the effect of corticosteroids on CA outcome in patients with refractory KD.

Bone mineral density in children and adolescents with systemic lupus erythematosus, juvenile dermatomyositis, and systemic vasculitis: relationship to disease duration, cumulative corticosteroid dose, calcium intake, and exercise.

OBJECTIVE: To describe the frequency of abnormal bone mineralization in a population of children with juvenile systemic lupus erythematosus (JSLE), juvenile dermatomyositis (JDM), and systemic vasculitis; and to investigate the relationship of bone mineral density (BMD) to cumulative corticosteroid dose, disease duration, Tanner stage, calcium intake, and exercise in these patients. METHODS: A retrospective chart review of children attending the pediatric rheumatology clinic at British Columbia's Children's Hospital was conducted to obtain demographic data (sex, ethnicity, disease duration, cumulative corticosteroid dose, and mean daily corticosteroid dose). All patients had at least one BMD measurement by dual energy x-ray absorptiometry (DEXA) at lumbar spine, hip, and total body. BMD was expressed as g/cm2 and Z scores; an abnormal Z score was defined as

The role of subcutaneous administration of methotrexate in children with juvenile idiopathic arthritis who have failed oral methotrexate.

OBJECTIVE: To describe the outcome of patients with juvenile idiopathic arthritis (JIA) treated with subcutaneous (Sc) methotrexate (MTX) after failing oral MTX (either because of inefficacy or toxicity) in a clinic population. METHODS: The study cohort was identified from our clinical database, and consisted of 61 children with JIA treated with MTX between 1988-2001. All patients fulfilled International League Against Rheumatism (ILAR) criteria for JIA and had disease duration of >/= 6 months and 3 or more active joints before institution of MTX. All patients had a core set of outcome variables assessed at baseline and at 3 months after achieving both maximum oral and SC MTX. Outcome variables included physician global assessment of disease activity, number of active joints, number of joints with limited range of motion, duration of early morning stiffness, and erythrocyte sedimentation rate (ESR). Improvement was defined as at least 30% improvement from baseline in 3 of 5 variables in the core set, with no more than one of the remaining variables worsening by more than 30%. RESULTS: A total of 61 patients, 43 females and 18 males with JIA were studied. The disease subtypes were systemic 8, polyarticular 25 (12 rheumatoid factor positive), oligoarticular 14, enthesitis related arthritis 5, and unclassified 4. Thirty-one patients were switched to SC MTX, 13 of whom had not improved, and 18 who had improved, but had nausea (11) or insufficient clinical improvement (7). After 3 months of SC MTX treatment, 76% of patients were classified as improved and 23% as not improved. Toxicity on SC MTX was less than on oral MTX. CONCLUSION: Our results suggest that for patients failing oral MTX either because of inefficacy or toxicity, the use of SC MTX has a high likelihood of success with more than 70% of patients achieving clinically significant improvement, without clinically significant toxicity.

Radiologic outcome and its relationship to functional disability in juvenile rheumatoid arthritis.

OBJECTIVE: To determine the radiologic outcome in juvenile rheumatoid arthritis (JRA) and the relationship of radiologically detected joint damage to functional disability using multivariate analyses. METHODS: Selection criteria included a diagnosis of JRA made by 1977 American College of Rheumatology criteria, onset of arthritis > or = 5 years prior to study, current age > or = 8 years, a minimum grade 3 reading ability, and the availability of radiographs. Disability was measured by the Childhood Health Assessment Questionnaire (CHAQ) and Steinbrocker classifications. Radiographs taken within 2 years after onset (early) and the most recent radiographs (late) were examined by a single pediatric radiologist blinded to patients' identities, diagnoses, and outcomes. Multiple regression analyses were performed. RESULTS: On late radiographs the frequencies of joint space narrowing were 38, 14, 43, and 79%, respectively, among patients with systemic, pauciarticular, rheumatoid factor (RF) negative polyarticular, and RF positive polyarticular onset; erosions occurred in 63, 25, 39, and 75%, respectively. Early erosions were most frequent in patients with RF+ polyarticular onset, while both joint space narrowing and erosions occurred early in systemic onset. Radiologic signs of joint damage were most frequent at hips and wrists, while knees and ankles were relatively spared. Based on patients who had radiographs performed within one year of clinical study, 17.7% of the variation in CHAQ score was explained by joint space narrowing, 32.4% by pain, and 5% by a severe rating on physician's global estimate of disease activity. The odds of a Steinbrocker class > I were increased by joint space narrowing, pain, systemic onset, and active joint count. CONCLUSION: Differences in the frequencies and patterns of joint damage occur both among JRA onset subtypes and among individual joints. Radiographic damage, especially joint space narrowing, correlates with functional disability. However, pain is the major contributor to variation in CHAQ scores.

Predictors of pain in children with established juvenile rheumatoid arthritis.

OBJECTIVE: To examine demographic and disease-related variables that affect pain in a large cohort of patients with juvenile rheumatoid arthritis (JRA). METHODS: Selection criteria were an onset of JRA >/=5 years prior to study and age >/=8 years at the time of the study. Pain was measured by a self-administered 10-cm visual analog scale. Possible explanatory variables studied included age at study, sex, race, onset subtype, active disease duration, active joint count, and physician's global assessment (PGA). RESULTS: In a multiple regression model, active disease duration, PGA, and age at study were independent predictors explaining 22% of the variation in pain scores. Stratified analyses showed an effect of age in the 8-15-year group, but not in older patients. CONCLUSION: Disease-related factors explain only a small proportion of the variation in pain scores. Age has an effect on pain scores only in younger patients. The role of other factors, including psychosocial factors, needs further study.

Disease course and outcome of juvenile rheumatoid arthritis in a multicenter cohort.

OBJECTIVE: To determine the disease course and outcome in a multicenter cohort of patients with juvenile rheumatoid arthritis (JRA). METHODS: All patients with JRA seen at 3 pediatric rheumatology centers were identified from databases and/or clinic records. Inclusion criteria were a diagnosis of JRA (1977 American College of Rheumatology criteria), a followup period of at least 5 years since onset, and a minimum age of 8 years. Patients were examined and completed a Childhood Health Assessment Questionnaire (CHAQ). Kaplan-Meier curves were constructed to estimate rates of remission, relapse, and arthroplasty. Remission was defined as absence of active arthritis while off treatment for at least 2 years. Outcome measures were active disease duration, CHAQ scores, pain determined by visual analog scales, physician's global assessments, and Steinbrocker functional classifications. Years of education and employment status were ascertained. RESULTS: We studied 392 patients of 652 (60%) who met the selection criteria. The probabilities of remission at 10 years after onset were 37, 47, 23, and 6% for patients with systemic, pauciarticular, RF- polyarticular, and RF+ polyarticular JRA, respectively. The probability of relapse varied from 30 to 100% at 15 years. The probability of arthroplasty varied from 13 to 57% after 15 years of active disease. We found 2.5% of patients assessed were in Steinbrocker Classes III or IV and 6% were in the highest CHAQ score (> 1.5) group. Compared with national statistics, fewer female patients received post-secondary education and unemployment rates for patients 20 to 24 years of age were higher. CONCLUSION: Our results indicate that JRA is a disease that often extends into adulthood. Compared to previous decades, functional outcome has improved; however, the estimated rate of arthroplasty remains very high. Patients with JRA may have difficulty entering the workforce.

Patterns of joint involvement at onset differentiate oligoarticular juvenile psoriatic arthritis from pauciarticular juvenile rheumatoid arthritis.

OBJECTIVE: To compare the patterns of joint involvement of patients with oligoarticular onset juvenile psoriatic arthritis (Oligo-JPsA) and pauciarticular onset juvenile rheumatoid arthritis (Pauci-JRA) in order to estimate the predictive performance of specific patterns for the diagnosis of Oligo-JPsA. METHODS: Twenty-three children who fulfilled the diagnostic criteria for JPsA (Vancouver criteria) and who had fewer than 5 joints involved in the first 6 months of disease (Oligo-JPsA), and 64 children with Pauci-JRA (ACR criteria) were enrolled. Patients were also classified with respect to the ILAR criteria for juvenile idiopathic arthritis (JIA). Patient characteristics and clinical features at onset and during followup were determined. Patterns of joint involvement at onset of disease and their ability to differentiate between Oligo-JPsA and Pauci-JRA/Oligo-JIA were evaluated. RESULTS: Small joint disease (defined as involvement of any of the metatarsophalangeal or proximal or distal interphalangeal joints of the foot, or metacarpophalangeal or proximal or distal interphalangeal joints of the hand) was significantly more frequent in Oligo-JPsA than in Pauci-JRA at disease onset. The odds of patients with Oligo-JPsA having small joint disease or wrist disease within 6 months of disease onset were much higher than those with Pauci-JRA or Oligo-JIA (p < 0.05 or 0.001). CONCLUSION: Small joint disease and wrist disease are suggestive of Oligo-JPsA. The use of a criterion consisting of small joint disease and/or wrist disease and/or dactylitis instead of dactylitis alone may increase the ability to differentiate Oligo-JPsA from Pauci-JRA or Oligo-JIA.

Guidelines for blood test monitoring of methotrexate toxicity in juvenile idiopathic arthritis.

OBJECTIVE: To assess the utility of the American College of Rheumatology guidelines for monitoring methotrexate (MTX)-related toxicity in a cohort of children with juvenile idiopathic arthritis (JIA). METHODS: Eighty-nine patients with JIA treated with MTX were monitored prospectively: aspartate aminotransferase (AST), alanine aminotransferase (ALT), complete blood count (CBC), and differential blood count were measured prior to starting MTX, and then monthly. Significantly abnormal blood tests (SABT) were prospectively defined as (1) significantly elevated liver enzymes (SELE) greater than twice the upper limit of normal; (2) granulocyte count < 1.5 109/l; (3) lymphocyte count < 0.9 109/l; or (4) hemoglobin decreased by > 2 g/l from previous level. Clinical interventions, current and cumulative MTX dose, duration of treatment, comorbidity, and concurrent medications at the time of the first SABT identification were recorded. Independent t tests and chi-squared tests were used for comparisons, and the probability of developing a SABT was calculated by Kaplan-Meier survival analysis. RESULTS: Forty percent of patients had a SABT: 26% had hematological abnormalities and 14% had SELE. Ninety-five percent of patients with SABT had symptoms consistent with a viral infection when the SABT was drawn and MTX dose was withheld until results had normalized on repeat testing. SABT persisting beyond one month occurred in only 2 patients, and their abnormalities resolved by 6 months with no specific identified cause; they resumed MTX at a later time without recurrence of SABT. There were no differences between patients with and without SABT with respect to current or cumulative MTX dose, duration of treatment, and concurrent medications at the time of the SABT. The probability of developing a SABT was estimated to be 11% at 3 months, compared to 10% probability of having an abnormal blood test by chance alone. CONCLUSION: Routine blood tests every 4 to 8 weeks in children with JIA are unnecessarily frequent.

Early predictors of longterm outcome in patients with juvenile rheumatoid arthritis: subset-specific correlations.

OBJECTIVE: To determine early predictors of longterm outcome in juvenile rheumatoid arthritis (JRA) in a multicenter cohort. METHODS: Patients were selected if they were > or = 8 years of age; the onset of arthritis occurred > or = 5 years before study; and a diagnosis of JRA was made at a participating center. Outcome variables were scores on self-administered Childhood Health Assessment Questionnaires (CHAQ) and active disease duration. Possible explanatory variables assessed included characteristics present at onset, HLA alleles, in particular the rheumatoid arthritis associated shared epitope (RASE), and radiographic indicators of joint damage within 2 years of onset. Data for 393 patients were available. Multivariate analyses were performed for the total group and for each onset subtype. RESULTS: Male sex correlated with worse disability in systemic onset JRA but less disability in RF negative, and a shorter active disease duration in RF positive polyarticular onset JRA. Positive antinuclear antibody correlated with a longer active disease duration in patients with pauciarticular onset JRA. Younger age at onset predicted longer active disease duration in pauciarticular and RF negative polyarticular, and a shorter active disease duration in systemic onset JRA. Residence on a reserve, rather than native North American race, correlated with worse disability. The RASE correlated with less disability in systemic JRA; but no correlation with outcome was evident for patients with rheumatoid factor positive polyarticular JRA. CONCLUSION: Variables predictive of longterm outcome in JRA are specific for each onset subtype. The most important early predictors were age at onset and sex of the patient. Place of residence may have a greater effect on disability than race. RASE may associate with a more favorable outcome in systemic onset disease.

Gender and ethnic origin have no effect on longterm outcome of childhood-onset systemic lupus erythematosus.

OBJECTIVE: To investigate the associations of gender and ethnic origin with longterm outcome in childhood-onset systemic lupus erythematosus (SLE). METHODS: The study cohort consisted of 51 patients (13 males and 38 females) with childhood-onset SLE followed for > or = 5 years at the British Columbia Children's Hospital in Vancouver. Fifteen patients were Caucasian, 14 Chinese, 9 East Indian, and 13 patients were of other ethnic backgrounds: none was African-American or Hispanic. Outcome measures assessed retrospectively included Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index score (SDI), SLE-related death, need for dialysis or renal transplantation, and use of intensive immunosuppressive therapy. A SDI > or = 2 was assigned as poor outcome. RESULTS: The median age at diagnosis was 10.8 years and the median duration of followup was 7.2 years. Five-year survival was 100%; 10-year survival was 85.7% (12/14 patients). The median SDI score at last followup was 2.0 (range 0-9); 2.0 for male, 1.5 for female; 2.0 for Caucasian and 2.03 for non-Caucasian patients. Twenty-six out of 51 patients (51%) had poor outcome (SDI score > 2). Three female patients required dialysis: 2 had subsequent renal transplants. Thirty patients received intensive immunosuppressive therapy. The SDI scores, mortality, and need for intensive immunosuppressive therapy were not influenced by either gender or ethnic origin. CONCLUSION: The median SDI score was high for this cohort with childhood-onset SLE. In contrast to other published data, no association of male gender and/or non-Caucasian ethnicity with poor outcome was found in our study cohort.

The early pattern of joint involvement predicts disease progression in children with oligoarticular (pauciarticular) juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate features during the first 6 months of disease that may be associated with a poor outcome as measured principally by extension to a polyarticular disease course in patients with oligoarticular-onset juvenile rheumatoid arthritis (oligo-JRA). METHODS: This study was a retrospective review of patients who fulfilled the American College of Rheumatology criteria for oligo-JRA, were followed up for at least 5 years, and did not have juvenile psoriatic arthritis, spondylarthropathy-like disease, or rheumatoid factor positivity. Data from the first 6 months of disease were collected. Continuous variables were dichotomized and then screened by univariate analysis for association with poor outcome at the last followup visit, as measured by extension of involvement (>4 accumulated involved joints) and by "clinically meaningful" extension (> or =10 accumulated joints). Variables significantly associated with this latter outcome, with the addition of disease duration as a confounding independent variable, were included in a multiple logistic regression analysis. The same variables were then examined in separate multiple logistic regression models to look at other measures of outcome, including use of disease-modifying antirheumatic drugs (DMARDs) at any time, erosive disease on radiographs, any remission of disease ever occurring, physician's global assessment of disease activity at the last visit, and disability as measured by the Childhood Health Assessment Questionnaire (C-HAQ)/HAQ. RESULTS: Of the 205 patients (160 of whom were female) studied for a median of 10.8 years (range 5-26.6 years), 39.5% developed extension to >4 joints and 17.6% developed arthritis in > or =10 joints. Using the logistic regression model, symmetric disease was predictive of all measures of poor outcome: extension to > or =10 joints (odds ratio [OR] 19.2), the need to use DMARDs (OR 11.5), radiographic demonstration of erosive disease (OR 4.73), inflammatory activity at last followup visit (OR 3.23), no remission of disease (OR 4.73), and disability as measured by a C-HAQ score >0.12 (OR 2.95). Ankle and/or wrist disease was predictive of extension (OR 6.61) and erosions (OR 3.59). Wrist disease alone was predictive of the need to use DMARDs (OR 5.87) and of inflammatory disease activity at the last followup visit (OR 4.01). An elevated erythrocyte sedimentation rate (ESR) was predictive of extension (OR 3.76), the need to use DMARDs (OR 6.47), and no remission of disease (OR 2.30). Disease duration was a confounding variable for extension (OR 1.18) and erosive disease (OR 1.19). CONCLUSION: The early presence of ankle and/or wrist disease, symmetric joint involvement, and an elevated ESR in a child with oligo-JRA indicates the likelihood of disease progression.