Prevalence and outcome of juvenile idiopathic arthritis-associated uveitis and relation to articular disease

OBJECTIVE: To determine prevalence and complications of juvenile idiopathic arthritis (JIA)-associated uveitis, and to evaluate risk factors for uveitis and its relation to articular disease. METHODS: Records of 309 patients with JIA (244 female, 65 male, mean age at onset 4.9 yrs) were retrospectively reviewed. Occurrence of uveitis and complications were assessed among oligoarticular-onset JIA (193 patients), polyarticular-onset JIA (66 patients), and systemic-onset JIA (50 patients). The presence of antinuclear antibodies (ANA) was determined in patients with oligoarticular-onset JIA. Therapy and relapses of uveitis and arthritis were recorded at each visit during the followup (mean followup 7.6 yrs). RESULTS: Sixty-two patients developed uveitis (20.1%); 57 patients had oligoarticular-, 3 polyarticular-, and 2 systemic-onset JIA. Uveitis was asymptomatic in 56/62 cases. Fifty-five of the 62 patients (88.7%) developed uveitis within 4 years from disease onset. In patients with oligoarticular-onset JIA, an early age at disease onset and the presence of ANA (p < 0.05) and DRB1*11 (p < 0.03) were the best predictors of uveitis, while a polyarticular course was not associated to uveitis (p > 0.05). Active arthritis was present at the first episode of uveitis in 46/62 patients. Forty-four of the 62 patients experienced relapses of uveitis: in 20/62, relapses were concomitant to arthritis relapses; in 24/62 relapses presented without active arthritis. Complications of uveitis developed in 35.5% of the patients (22/62), leading to visual impairment in 13 patients. CONCLUSION: Current guidelines provide early identification of uveitis in 90% of patients. With the exception of the first episode of uveitis, uveitis and arthritis seem to run different courses; close ophthalmologic scrutiny then should also be maintained during arthritis remission.     

Temporomandibular involvement in juvenile idiopathic arthritis

OBJECTIVE: To study occurrence as well as clinical signs and symptoms of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) in a population representing all subtypes of JIA. METHODS: Ninety-seven consecutive children with JIA underwent orthodontic evaluation including an orthopantomogram (OPG). Further evaluation included patient characteristics, disease onset, course, and medical treatment. RESULTS: Forty-five percent of all children had TMJ involvement. Frequencies according to JIA subtypes: systemic 67%, oligoarticular (persistent and extended) 39%, rheumatoid factor (RF) negative polyarticular 59%, RF positive polyarticular 33%, enthesitis related arthritis 13%, psoriatic arthritis 33%, and other arthritis 50%. In children with a polyarticular course, irrespective of their disease onset, TMJ involvement was more frequent (55% vs 31% in oligoarticular course). In children with disease onset at a young age and/or an extended course of the disease, TMJ involvement was also more frequent. Pain during jaw excursion, absence of translation, asymmetry during maximal opening and protrusion, as well as crepitation during evaluation are predictors for TMJ involvement with a good specificity but a low sensitivity. Not all patients with TMJ involvement have clinical signs. CONCLUSION: Because of the high prevalence and discrepancy between clinical signs and presence of arthritis of the TMJ, regular orthodontic evaluation and OPG is recommended to recognize TMJ involvement and enable early intervention.     

Positive family history of psoriasis does not affect the clinical expression and course of juvenile idiopathic arthritis patients with oligoarthritis

OBJECTIVE: In the 1997 revision of the International League of Associations for Rheumatology (ILAR) criteria for juvenile idiopathic arthritis (JIA), a family history of psoriasis is an exclusion for the oligoarthritis category. We investigated whether psoriasis in a first or second degree relative influences the clinical expression and course of JIA patients with oligoarthritis. METHODS: In a cross-sectional study, consecutive oligoarticular-onset JIA patients were investigated. Clinical evaluations included confirmation of a family history of psoriasis and assessment of nail abnormalities, dactylitis, psoriatic rash, variables of JIA activity, and laboratory indicators of inflammation. Retrospective assessments included sex, onset age, disease duration, antinuclear antibodies, HLA-B27, uveitis, ocular complications, second-line therapies, intraarticular corticosteroid injections, radiographic joint lesions, joint involvement over time, and laboratory investigations at disease presentation and first observation. RESULTS: A total of 185 patients were included. Thirty-three had a positive family history of psoriasis (group 2) and 139 did not (group 1). Thirteen patients fulfilled the ILAR criteria for juvenile psoriatic arthritis (group 3). Patients in groups 1 and 2 were comparable for all parameters, except for a higher frequency of females in group 1 (P = 0.04). As compared with group 2, patients in group 3 were less frequently antinuclear antibody positive and had a more severe arthritis and a different distribution of joint involvement. CONCLUSION: We found close similarities in the clinical features and course among patients with oligoarthritis who had a positive family history for psoriasis and those who did not. These findings argue against the exclusion of the former patients from the oligoarthritis category of JIA.     

Outcome following onset of juvenile idiopathic inflammatory arthritis: II. predictors of outcome in juvenile arthritis

OBJECTIVE: To assess the relative contributions of demographic, clinical and laboratory variables in predicting outcome in juvenile idiopathic inflammatory arthritis (JIA), based on a review of the existing literature. METHODS: Electronic reference database searches for the previous 10 yr were conducted and studies examining the role of major potential predictors of main outcomes were identified. Where possible, subjects were grouped by JIA disease subtype. In addition to demographic variables, the following disease-related predictors were assessed: nature of joint involvement, acute-phase response, and presence of autoantibodies. These were then analysed for three main outcomes of interest: remission as assessed by disease activity; functional impairment; and structural damage as assessed by radiological joint erosions. RESULTS: In general, female gender, polyarticular and symmetrical joint involvement, elevated inflammatory markers and rheumatoid factor positivity were the most consistent predictors of a poor outcome, although the studies were frequently inconsistent in both the direction and the magnitude of the effects. CONCLUSIONS: These data are too variable to accurately identify those predictors associated with poor outcome following the onset of JIA. Although some of this variation may be the result of true differences between study populations, the vast majority of inconsistencies are explainable by the absence of standardized classification systems, outcome definitions, therapeutic approach and research tools. More comprehensive prospective evaluation is required before robust prediction models can be generated.     

Predictors of disease course and remission in systemic juvenile idiopathic arthritis: significance of early clinical and laboratory features

OBJECTIVE: To determine whether the disease course in systemic juvenile idiopathic arthritis (JIA) can be characterized as monophasic, polycyclic, or persistent, and to determine whether early clinical and laboratory characteristics can be used to predict the disease course and time to remission. METHODS: Forty-five children with systemic JIA diagnosed between 1996 and 2000 were followed up with a standardized data collection protocol, including data on clinical and laboratory features (mean followup 4.9 years). Disease was considered inactive if the clinical and laboratory features were normal. Three definitions of remission were applied to classify disease course. Predictors of disease course were evaluated using multiple logistic regression. Predictors of time to remission were evaluated using Cox proportional hazards regression. RESULTS: When applying a definition of remission requiring inactive disease while not receiving any medications for a period of 3 months, 42.2%, 6.7%, and 51.1% of the patients were classified as having monophasic, polycyclic, and persistent disease, respectively. Fever and active arthritis at 3 months (R2 = 0.42, area under the receiver operating characteristics curve [AUC] = 0.76) and an erythrocyte sedimentation rate (ESR) >26 mm/hour and corticosteroid use at 6 months (R2 = 0.49, AUC = 0.92) were predictive of a nonmonophasic course. Absence of active arthritis, an ESR of <26 mm/hour, and no requirement for corticosteroid therapy at 3 and 6 months were predictors of an earlier time to remission. CONCLUSION: The disease course in systemic JIA can be characterized as monophasic, polycyclic, or persistent using a definition of remission requiring 3 months of inactive disease while not receiving any therapy. Features at 3 and 6 months are predictive of the disease course and time to remission.     

Early predictors of severe course of uveitis in oligoarticular juvenile idiopathic arthritis

OBJECTIVE: To determine whether demographic, clinical, and laboratory variables at onset of arthritis can predict the development and the severity of anterior uveitis (AU) in oligoarticular juvenile idiopathic arthritis (JIA). METHODS: In a retrospective study, a cohort of 366 patients with oligoarticular onset JIA from 3 pediatric rheumatology centers were evaluated. Patients were classified in 3 groups: severe uveitis (SU) with a mean >/= 2 uveitis relapses/year with complications or need for immunosuppressive therapy; mild uveitis (MU) with a mean 相似文献   

Therapeutic use of etanercept in polyarticular course juvenile idiopathic arthritis over a two year period

OBJECTIVE: To analyse the treatment response to etanercept in patients with polyarticular course juvenile idiopathic arthritis (JIA). METHODS: 22 patients with polyarticular course JIA (19 females, three males; mean age 13.9 years; mean disease duration 6.3 years; 15 with polyarticular onset, seven with systemic onset, one with residual systemic activity; eight rheumatoid factor positive; eight with erosive disease) were treated with etanercept for up to 24 months. Etanercept was given subcutaneously at 0.4 mg/kg twice a week. Treatment response was ascertained in an open prospective study. RESULTS: All patients showed impressive clinical improvement, with a decrease in swollen joint count by an average of 10.1 joints (mean of 49% decrease), a decrease in tender joint count by 9.3 joints (mean of 94%), and decrease in total joint count by 11.2 joints (mean of 48%). Duration of morning stiffness decreased to less than 10 minutes. Furthermore, haemoglobin concentration increased on average by 14 g/l (mean of 15.3%) and packed cell volume increased by 0.035 (mean increase of 12%), and erythrocyte sedimentation rate decreased on average by 42.8 mm/1st h (mean decrease of 64%). No major side effects were noted. CONCLUSION: Etanercept continues to be clinically effective and well tolerated in patients with polyarticular course JIA over a two year period.     

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.     

Hip involvement in juvenile idiopathic arthritis

We analyzed the clinical, biological, and radiological aspects of hip involvement in juvenile idiopathic arthritis (JIA) in a developing country. The recruited patients fulfilled the International League Against Rheumatism criteria for the diagnosis of the JIA. Clinical, biological, and radiological parameters relating to the JIA were collected. Hip involvement was assessed according to clinical and radiological data related to hip disease. One hundred twenty-one patients were included (68 girls and 53 boys). The mean age of the disease onset was 9 ± 4.2 years (1–16 years).The mean age of the patients at the time of the study was 15 ± 10 years (2–46 years). The duration of the disease was 5 ± 8.5 years (0.5–39 years). Forty cases (33%) of the hip involvement were noted. The mean age was 24 ± 10.03 years (3–46 years); the sex ratio was 1:3. The mean duration of the hip disease was 0.6 ± 3.6 years (3–14 years). Hip arthritis seemed to be more frequent in polyarticular and enthesitis-related arthritis. The severity of the hip involvement was significantly correlated with early disease onset, disease duration, subtypes, and high disability (for all these data p < 0.05). This study suggested that in JIA hip involvement was more frequent in enthesitis-related arthritis and polyarticular subtypes. It was correlated with the severity and the early disease onset of the JIA, which was similar to reported data.     

Diagnostic accuracy of anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis

OBJECTIVE: To correlate serum anti-cyclic citrullinated peptide antibodies (anti-CCP) levels with juvenile idiopathic arthritis (JIA) subtypes and with an erosive disease course. METHODS: The study group comprised 122 children with JIA; 16 were evaluated during both active disease and remission. Nineteen children with systemic lupus erythematosus (SLE), 27 with rheumatoid arthritis (RA), and 15 healthy children were also included in the study. Twelve children with JIA were rheumatoid factor (RF) positive, and 34 patients had persistent erosive joint disease. Anti-CCP antibody levels were determined by ELISA; values above 5 relative units were regarded as positive. RESULTS: Three girls with seropositive polyarticular JIA and erosive joint disease had positive anti-CCP values. Children evaluated during active disease and remission, patients with SLE, and healthy children all had negative anti-CCP antibody levels. However, 19/27 (70%) adult patients with RA had positive anti-CCP antibody values. CONCLUSIONS: In contrast with RA, anti-CCP positivity is only rarely found in patients with JIA. In patients with RF positivity and/or in patients with erosive joint disease, anti-CCP can be detected.     

Prevalence and complications of uveitis in juvenile idiopathic arthritis in a population-based nation-wide study in Germany: suggested modification of the current screening guidelines

OBJECTIVES: To analyse the prevalence and complications of uveitis and their predictors in a large cohort of patients with juvenile idiopathic arthritis (JIA). METHODS: Data of 3271 JIA patients as classified by International League of Associations for Rheumatology (ILAR) criteria included in a national database during 1 yr were analysed. RESULTS: Uveitis prevalence was 12% of all JIA patients. The most frequent were oligoarthritis extended (25%) and persistent (16%). JIA patients with uveitis were significantly younger at onset of arthritis (3.8 vs 7.0 yrs) or ANA-positive (86% vs 42%) than the patients without uveitis. Predictors of uveitis included age at onset (P= 0.03) and ANA-positivity (P< 0.01) besides the presence of a certain JIA subgroup (P= 0.04). Uveitis was clinically silent in 75% of the oligoarthritis but in none of the enthesitis-related arthritis patients. The median onset of uveitis was 5.5 months after arthritis manifestation. In 73%, 77% and 90%, uveitis developed within 1, 2 and 4 yrs after arthritis, respectively. Anterior uveitis was the most common anatomic type of uveitis (83%). Uveitis complications at mean follow-up of 5.6 yrs were common (56%), and predictors for complications included presence of complications at first visit (P< 0.001) and uveitis manifestation before arthritis (P= 0.001), but not ANA positivity. CONCLUSIONS: The JIA subgroups markedly differ with respect to the prevalence and course of associated uveitis. Ophthalmological screening should be initiated early after arthritis onset and the intervals be related to the JIA subgroup. A modification of the current screening guidelines is suggested.     

Circulating survivin indicates severe course of juvenile idiopathic arthritis

BACKGROUND: Survivin is an anti-apoptotic protein that has been recently suggested as a predictive marker of joint destruction in adult rheumatoid arthritis. We assessed the presence of extracellular survivin in patients with juvenile idiopathic arthritis (JIA). METHODS: Survivin levels were assessed in the circulation of 46 patients with JIA and in the age- and gender-matched controls (n=46) having no inflammatory disease, by ELISA. Survivin levels were analyzed with respect to the onset type and the activity of the joint disease. The intensity of inflammation and cartilage turnover was measured as levels of IL-6, serum amyloid A protein (SAA), and cartilage oligomeric matrix protein (COMP), respectively. RESULTS: The levels of extracellular survivin were significantly higher in JIA compared to the controls (p=0.0002). High levels of survivin (above mean + 2SD of the controls) were detected in 8/46 (17% JIA patients. High survivin expression was associated with polyarticular onset, active phase of arthritis. In contrast, survivin was neither related to the levels of IL-6, SAA, nor to COMP. CONCLUSION: Circulating survivin is expressed in a significant group of patients with JIA being associated to a severe course of the disease. It may be potentially used to select children with unfavorable prognosis of JIA who are in need of active pharmacologic treatment.     

Resorption of the temporomandibular condylar bone according to subtypes of juvenile chronic arthritis

OBJECTIVE: To evaluate the relative impact of sex, type of onset, course of disease, age at onset, duration of disease and status of HLA-B27, antinuclear antibodies (ANA), and rheumatoid factor on the risk of developing a condylar erosion. METHODS: Condylar changes of the temporomandibular joint (TMJ) were diagnosed on orthopantomograms from 169 consecutive patients with juvenile chronic arthritis (JCA). A multiple regression analysis was applied to establish the relative weight of the independent variables affecting the severity of the condylar erosion. RESULTS: It was found that 62.1% of the patients exhibited condylar resorption. The highest prevalence was seen in children with a polyarticular onset or course of disease and early age at onset and severe resorption was also frequent in these groups. Patients with positive ANA also had a high prevalence but with a mild degree of resorption. In contrast, HLA-B27 positive patients had a lower risk of TMJ involvement and resorptive changes of the condyle. CONCLUSION: Polyarticular and early onset arthritis are associated with a high risk for TMJ involvement and a severe condylar bone loss can be expected. ANA positive patients have a high prevalence, and B27 positive patients have a low prevalence of TMJ arthritis but in both subgroups, the outcome of the bone resorptive process is mild.     

Etanercept and uveitis in patients with juvenile idiopathic arthritis

OBJECTIVES: Etanercept has been shown to be effective for the treatment of juvenile idiopathic arthritis (JIA). The therapeutic efficacy of etanercept for chronic uveitis, a major complication of JIA, has not been evaluated so far. Therefore, the appearance of chronic anterior uveitis and associated complications in JIA patients treated with etanercept was evaluated. METHODS: Questionnaires were sent to paediatric rheumatologists treating a total of 310 JIA patients with etanercept. RESULTS: Two hundred and twenty-nine questionnaires (74%) were returned. Before institution of etanercept, 31 patients (13.5%) had a history of uveitis with a total of 102 flares. Twenty-eight patients belonged to the high-risk groups of the oligoarticular and seronegative polyarticular subtypes. Upon commencing etanercept, 32 courses of uveitis occurred in 19 patients and in two further patients (1%) in whom uveitis occurred for the first time. Twenty of them belonged to the high-risk group. Uveitis during etanercept therapy occurred in 12 of 15 patients (80%) with more than one course of uveitis, and in seven of 16 patients (44%) with only one course before etanercept therapy. Complications were noted in 12 patients before and in eight during etanercept treatment. In 87% of the uveitis patients, arthritis demonstrated a significant or complete response. CONCLUSION: During treatment with etanercept, there were both relapses and first courses of uveitis. In addition, the frequency and severity of uveitis seemed not to be influenced by etanercept. In particular, patients with relapsing uveitis before institution of etanercept treatment remain at high risk of the development of uveitis flares despite etanercept treatment.     

Remission in Juvenile Idiopathic Arthritis: Current Facts

Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritic disease affecting children. Despite the availability of potent disease-modifying antirheumatic medications, most children still experience a chronic course with long periods of active disease. Goals of treatment should include achievement of disease remission with optimal physical functioning that allows children to lead normal lives with no structural joint damage. The term remission implies a complete lack of disease activity. This article focuses on recently developed preliminary criteria for inactive disease and remission in JIA. Recent studies using these new definitions demonstrate only modest rates of achievement of remission favoring children with persistent oligoarticular JIA. Children with rheumatoid factor-positive polyarticular JIA are least likely to achieve remission. Therapeutic strategies to achieve remission are also discussed.     

Low density neutrophils in patients with juvenile idiopathic arthritis.

OBJECTIVES: (1) To study the correlation among conventional clinical and laboratory parameters and the relation between the number of lymphocytes and neutrophils (L/N) in cell suspensions from peripheral blood of patients with juvenile idiopathic arthritis (JIA). (2) To evaluate the L/N relation of JIA patients after an 8 year follow-up period. METHODS: Fifty-one JIA patients (25 female, disease course: 19 systemic, 15 polyarticular, 17 pauciarticular) were enrolled in the study. To measure the L/N relation, we used Boyum's method: The leucocyte separation was done by centrifugation of peripheral blood on Ficoll-Hypaque (FH) gradient, and the number of lymphocytes, monocytes, and neutrophils in 500 cells was determined. The following clinical and laboratory parameters were evaluated: disease activity, number of active and limited joints, functional capacity, erythrocyte sedimentation rate (ESR), and C reactive protein (CRP). Twenty-four healthy children were used as controls. We also studied 13/51 patients from our Pediatric Rheumatology Unit who had been evaluated by the same method 8 years before. RESULTS: We observed the lowest L/N relation in patients with active disease, especially those with polyarticular course. A statistical correlation was also observed with the acute-phase reactants (ESR and CRP, p < 0.05). The majority of patients who had presented a low L/N relation at the first evaluation (8 years before) had a worse outcome. CONCLUSION: The measure of L/N relation from peripheral blood could be used as an auxiliary tool in the assessment of the activity and outcome of JIA patients, especially at disease onset.     

Do patients with juvenile idiopathic arthritis in remission exhibit active synovitis on joint ultrasound?

The aim of the study was to assess the presence and characteristics of subclinical synovitis using power Doppler (PD) ultrasonography on patients with juvenile idiopathic arthritis (JIA) in clinical remission and compare the findings with those of healthy children. A cross-sectional study was carried out involving the clinical (physical exam, functional capacity and laboratory tests) and ultrasonography evaluation of 34 joints (synovial fluid/hypertrophy, PD signal and bone erosion). Subclinical synovitis was defined as the presence of synovial hypertrophy/joint effusion with or without any PD signal. Thirty-six patients (11.5 ± 3.74 years) and 36 controls (sex and age matched) were evaluated (2,448 joints). Twenty-seven patients were in remission on medication (mean duration: 1.8 ± 2.2 years). Subclinical synovitis was detected in 41.7 % patients and 11.1 % controls (p = 0.003). Erosion was detected in three patients (8.3 %). Subclinical synovitis was found in 38/1,224 (3.1 %) joints in the patients (most affected: radiocarpal wrist, anterior elbow and tibiotalar ankle) and 8/1,224 (0.6 %) joints in the controls (most affected: radiocarpal wrist). Differences in subclinical synovitis between patients and controls were found in the elbows (p = 0.033) and ankles (p = 0.006). A greater frequency of subclinical synovitis was found in patients with the extended oligoarticular or polyarticular subtypes (p = 0.013), those at an older age at disease onset (p = 0.007) and using methotrexate (p = 0.049). Patients with JIA in remission exhibit subclinical synovitis more frequently than controls. Subclinical synovitis was more frequent in patients with the polyarticular involvement and those at an older age at disease onset.     

Patients with antinuclear antibody-positive juvenile idiopathic arthritis constitute a homogeneous subgroup irrespective of the course of joint disease

OBJECTIVE: We recently hypothesized that in the International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA), the presumably homogeneous patient group characterized by early onset of disease, a female predilection, the presence of antinuclear antibodies (ANA), asymmetric arthritis, and the risk for iridocyclitis is classified into different categories. We sought to investigate whether ANA-positive patients belonging to the ILAR categories of oligoarthritis and rheumatoid factor (RF)-negative polyarthritis share homogeneous features and to compare these features with those of ANA-negative patients with JIA in the same categories. METHODS: We identified patients who were followed up during a 15-year period. All patients had JIA according to the ILAR criteria, with oligoarticular or polyarticular onset. ANA positivity was defined as 2 or more positive results at a titer of >or=1:160. Demographic and clinical features, including the number of joints involved over time and measures of JIA severity at the last followup visit, were recorded retrospectively. RESULTS: A total of 256 patients were included: 190 were ANA positive (109 had persistent oligoarthritis, 48 had extended oligoarthritis, and 33 had RF-negative polyarthritis), and 66 were ANA negative (35 had RF-negative polyarthritis, and 31 had oligoarthritis). All patients who were positive for ANA were similar in terms of age at disease presentation, female-to-male ratio, and frequency of symmetric arthritis and iridocyclitis. Compared with ANA-positive patients with polyarticular disease, ANA-negative patients with polyarticular arthritis were older at disease presentation and had a lower frequency of iridocyclitis, a higher frequency of symmetric arthritis, a greater cumulative number of joints affected over time, and a different pattern of joint disease, with a greater frequency of shoulder and hip involvement. The strong relationship between the presence of ANA and younger age at disease presentation, asymmetric arthritis, and development of iridocyclitis was confirmed by multivariate regression analysis. CONCLUSION: Our results support the hypothesis that patients with similar characteristics are currently classified into different JIA categories. The value of ANA positivity as a possible modifier of the current classification system deserves consideration.     

Methotrexate is an effective treatment for chronic uveitis associated with juvenile idiopathic arthritis

OBJECTIVE: To assess the effectiveness of methotrexate (MTX) in the treatment of juvenile idiopathic arthritis (JIA) associated uveitis, which is still one of the most common causes of visual impairment. METHODS: A retrospective chart review of patients with the diagnosis of uveitis associated with JIA between July 1, 2002, and December 31, 2002. RESULTS: Four hundred sixty-seven patients with JIA were followed. Thirty-eight had uveitis: 31 associated with oligoarticular JIA and 7 with psoriatic JIA. Twenty-five of the 38 patients received MTX; in 23 patients uveitis was the indication for MTX therapy. In the MTX treated group 46/50 eyes had uveitis, the mean (range) age at onset of uveitis was 7.82 years (1.8-15.8), and the mean age at onset of arthritis was 7.25 years (1.25-15.7). MTX treatment was started an average of 11.4 months (0-72) after the onset of uveitis. The mean MTX dose was 15.6 mg/m2. Remission occurred after 4.25 months (1-12). Mean duration of remission was 10.3 months (3-27). The total duration of MTX therapy was 661 months and patients were in remission for 417/661 months. In 6 patients MTX was discontinued after 12 months of remission. Four patients were still in remission after 7.5 months (1-14). CONCLUSION: MTX seems to be an effective therapy for JIA associated uveitis.     

The influence of patient characteristics, disease variables, and HLA alleles on the development of radiographically evident sacroiliitis in juvenile idiopathic arthritis

OBJECTIVE: To assess the frequency of sacroiliitis and the radiographic and clinical outcome in juvenile idiopathic arthritis (JIA) and determine patient characteristics, early disease variables, and genetic markers that predict development of sacroiliitis. METHODS: We performed a retrospective cohort study of 314 (79%) of the 400 JIA patients first admitted to the hospital between 1980 and 1985. The participants were examined after a median disease duration of 14.9 years (range 11.7-25.1). Radiographs of the sacroiliac joints, hips, ankles, and tarsi were obtained and studied in a blinded manner by 2 radiologists. The presence of HLA-DRB1 and DPB1 alleles was determined by genotyping and that of HLA-B27 by serologic testing. Variables relating to the onset and course of the disease were obtained by chart reviews. RESULTS: Twenty (6%) of the JIA patients developed radiographic sacroiliitis according to the New York criteria. In 9 patients (45%), sacroiliitis had not been demonstrated before the followup examination. At followup, spinal flexion (lateral and anterior) was reduced in 70-75% of patients with sacroiliitis and in 30-35% of those without sacroiliitis. Compared with the JIA patients without sacroiliitis, those with sacroiliitis more frequently had inflammatory back pain, enthesitis, radiographic changes in the hips and calcanei, erosions of any peripheral joint, and uveitis. Predictors of sacroiliitis were HLA-B27, absence of DPB1*02, hip joint involvement within the first 6 months, and disease onset after age 8 years. The following factors were more common among patients in whom sacroiliitis developed than in other JIA patients: DRB1*04, male sex, family history of ankylosing spondylitis, psoriasis, inflammatory back pain, and enthesitis within the first 6 months. CONCLUSION: In the current study, radiographically evident sacroiliitis had developed in 6% of JIA patients after a median disease duration of 14.9 years. HLA-B27, absence of DPB1*02, late onset of disease, and early hip involvement were predictors of sacroiliitis.