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1.
OBJECTIVE: To identify features of the T cell receptors (TCRs) present on clonally expanded T cells in the joints of patients with similar types of childhood rheumatic disease. Vbeta8 and Vbeta20 TCRs were selected as prototypic for polyarticular juvenile rheumatoid arthritis (JRA) and pauciarticular/juvenile spondylarthropathy (SpA), respectively. METHODS: The portion of the TCR beta chain involved in antigen recognition in the synovial tissue, synovial fluid, and peripheral blood from patients with JRA and juvenile SpA was cloned and sequenced. The frequency of expanded clonotypes, size of expansions, the Jbeta region, and sequence motifs were determined for >2,000 sequences. RESULTS: The majority of Vbeta20 and Vbeta8 clonal expansions were found in the joint rather than the peripheral blood. While instances of both Vbeta8 and Vbeta20 clonal expansion were detected in all disease types, the features of these expanded clonotypes were specific for disease type and Vbeta family. For example, Vbeta20 clonal expansion was characterized by many small expanded clonotypes in samples from patients with pauciarticular JRA and juvenile SpA while single large Vbeta8-specific expansions were found only in patients with polyarticular disease. Motifs specific to individual patients were identified, and for Vbeta20 clonotypes, a motif was found in synovial tissue samples. CONCLUSION: Identification of common TCR features in oligoclonal expansions within individual patients and between patients with the same type of JRA suggests the recognition of a common or limited group of antigens in these diseases.  相似文献   

2.
OBJECTIVE: To describe outcome and determine predictive factors in juvenile rheumatoid arthritis (JRA) and juvenile spondyloarthopathy (JSpA). METHODS: Seventy-two children with chronic arthritis were studied on first admission to the pediatric rheumatology clinic and after a mean of 9.7 +/- 1.8 yrs of disease duration. RESULTS: At followup, 53 patients had JRA and 19 had JSpA. Eleven (21%) of the patients with JRA did not meet the criteria for JRA on first admission, and 12 (22%) of 54 children diagnosed as having JRA on first admission were later reclassified as having another disease. Remission occurred in 43 (60%) of the 72 patients with JRA and JSpA. Forty-four patients (60%) reported no disability by the childhood or adult Health Assessment Questionnaire and 18 patients (25%) had developed joint erosions. Disease modifying antirheumatic drugs (DMARD) were given to 49 patients (68%) after a median of 0.8 yrs (range 0.2-10.8) disease duration. The patients who developed erosions and disability tended to have started treatment later than those who did not (NS). Active disease 5 years after onset was a predictor of disability in JRA and JSpA (OR 4.5, 95% CI 1.6-12.5). Predictors of joint erosions were long duration of elevated erythrocyte sedimentation rate (ESR) (OR 3.7/yr of elevated ESR, 95% CI 1.9-7.2), long disease duration before first admission (OR 1.5/yr of duration, 95% CI 1.1-2.1), long disease duration before treatment with DMARD (OR 1.8, 95% CI 1.0-3.3), and IgM rheumatoid factor (OR 12 x 10(4), 95% CI 0-1.2 x 10[52]). CONCLUSION: The longterm outcome in JRA and JSpA was more favorable than previously reported. This may be explained by less selection in favor of severely diseased patients and by the use of early aggressive treatment regimes.  相似文献   

3.
OBJECTIVE: To describe the demographics and clinical disease in affected sibpairs (ASPs) with juvenile rheumatoid arthritis (JRA), and to compare JRA as it occurs in ASPs with that from non-ASP JRA populations described in the literature. METHODS: A rare disease research registry was established with a focus on JRA ASPs to facilitate accrual of patients for genetic, epidemiologic, and clinical studies. Physicians likely to care for patients with JRA were made aware of the registry and its goals by a variety of methods and asked to refer patients for entry. RESULTS: To date, 71 ASPs have been registered and complete information has been obtained. These affected sibs differed in age by a mean of 4.1 years (SD 3.4) and in age at disease onset by 2.8 years (SD 3.0). The actual time difference between onset in sib 1 versus sib 2 averaged 4.4 years (SD 4.2). Sixty-three percent of the sibpairs were concordant for sex, and 76% for JRA onset type. Onset type within sibpairs did not appear to be random, based upon comparisons with non-ASP populations. Greater than expected concordance was seen among those with pauciarticular-onset and polyarticular-onset JRA. Seventy-nine percent of the pairs were concordant for course type. Seven sets of twins were included (approximately 10% of the total), all were concordant for onset and course type (6 sets with pauciarticular, 1 set with polyarticular), and disease onset was separated by a mean of only 3.3 months. Within the onset and course types, the clinical disease, such as the female:male ratio, age at onset, and serologic findings, in ASPs resembled that which has been described in the literature. CONCLUSION: A higher than expected degree of concordance for onset type of JRA exists between sibpairs, indicating that genetic influences play a role. Affected sibs do not tend to develop their disease at approximately the same point in time, except for the twin sets. Clinical features of the disease within the various subtypes appear similar to those in non-ASP populations.  相似文献   

4.
5.
OBJECTIVE: It has been suggested that double negative (CD4-CD8-) (DN) and gamma/delta T cells may be involved in some autoimmune diseases. We investigated peripheral blood DN and gamma/delta T cell levels in patients with active juvenile rheumatoid arthritis (JRA). METHODS: DN and gamma/delta T cell levels were measured in 42 patients with active JRA and in 10 healthy controls comparable for age by an immunofluorescence double staining procedure. RESULTS: All 3 JRA onset types had DN and gamma/delta T cell levels not significantly different from those of controls, although a wide scattering of data was present. No correlation was found between DN or gamma/delta T cell levels and erythrocyte sedimentation rate values or the number of active joints. When patients were divided according to treatment, we found that DN and gamma/delta T cell levels were significantly lower (p = 0.001, p = 0.02, respectively) in patients receiving methotrexate (MTX) than in patients not receiving MTX. The association of MTX treatment with a decrease in DN and gamma/delta T cell levels was also confirmed in a followup study of individual patients. Among patients not receiving MTX, patients with systemic JRA presented DN T cell levels significantly higher than those of controls. In 5 patients with pauciarticular JRA DN and gamma/delta T cell levels were higher in synovial fluid than in the peripheral blood. CONCLUSIONS: We found an increase in peripheral blood DN T cell levels in systemic JRA; treatment with MTX appears to be associated with a decrease in DN and gamma/delta T cell levels.  相似文献   

6.
Early studies showed few immunologic abnormalities in juvenile rheumatoid arthritis (JRA) patients. There were no specific laboratory markers useful for diagnosis and assessment of the course of disease in JRA. Previous work showed an association of antinuclear antibodies (ANA) with early-onset pauciarticular disease and iridocyclitis. Similarly, the presence of 19S immunoglobulin (Ig) M rheumatoid factors (RF) was associated with late-onset polyarticular disease in girls. More recent studies have detected many unique autoantibodies. Newer assays show 19S IgM RF in up to 35% of JRA patients, although still mainly in girls with late-onset polyarticular disease. Hidden 19S IgM RF can be shown in up to 75% of JRA patients using different procedures, primarily in those with active polyarticular-or pauciarticular-onset disease. Immune complexes have been detected in JRA patients by means of different techniques; their presence usually correlates with active disease. Studies on a specific ANA in JRA have shown no common extractable nuclear antigen, but antihistone antibodies have been found in up to 75% of cases, again mainly in those with pauciarticular onset and iritis. Finally, a variety of unusual immunologic proteins have also been detected, including anti-ocular, anti-cellular, anti-cardiolipin, anti-perinuclear factor, and anti-collagen antibodies. This review evaluates the significance of these antibodies that can now be found in JRA.  相似文献   

7.
OBJECTIVE: To examine racial differences in disease expression in African American and Caucasian children with pauciarticular and polyarticular juvenile rheumatoid arthritis (JRA). METHODS: A retrospective chart review was conducted of 35 African American and 137 Caucasian children with pauciarticular and polyarticular JRA. RESULTS: African American children were significantly older than Caucasian children at the time of presentation. This was true both for the group as a whole and for each of the disease onset subtypes. African American children were less likely to have positive antinuclear antibody tests than Caucasian children. This finding paralleled a low incidence of uveitis in African American children. African American children were also more likely to have IgM rheumatoid factors (detected by latex agglutination) than Caucasian children. This was true even for African American children with pauciarticular JRA. CONCLUSION: There are significant phenotypic differences between African American and Caucasian children with JRA.  相似文献   

8.
OBJECTIVES: To translate into Argentine Spanish and cross-culturally adapt the Childhood Health Assessment Questionnaire (CHAQ) and validate the adapted instrument in Argentine patients with juvenile rheumatoid arthritis (JRA). METHODS: Five bilingual Argentine pediatric rheumatologists translated into Argentine Spanish and cross-culturally adapted the United States English CHAQ. Pretesting was done in a sample of 23 parents using a probe question technique. Parents of 70 patients with JRA and 21 healthy children (controls) participated in the validation phase. All were from Argentina. RESULTS: The mean disability index (DI) scores for patients with systemic, polyarticular, or pauciarticular onset JRA were 0.64, 0.32, and 0.1, respectively. Healthy controls averaged 0.2. Intercomponent correlations were between 0.4 and 0.9, suggesting internal consistency, but also some redundancy. Test-retest reliability, studied at a 1-week interval, was moderate (mean DI 0.44 [in clinic] and 0.29 [one week later], Pearson's correlation = 0.82). We compared CHAQ scores from 15 parents with those of their children > 10 years of age. Significantly higher DI scores were given by patients than their respective parents (P > 0.019), but the pairwise scores (parent-patient) were highly correlated (r = 0.986). CONCLUSIONS: Cross-cultural adaptation of the US CHAQ to Argentina required few changes. Although DI scores for all patient subgroups were higher than for controls subjects, the scores were low, particularly for those with pauciarticular disease. Prospective studies designed to examine the sensitivity to change and predictive validity will help to assess further the usefulness of the adapted CHAQ in the Argentine population.  相似文献   

9.
Rheumatoid arthritis (RA) is an inflammatory disease of the synovium which may lead to proliferative and degenerative changes in the body's joints, including the temporomandibular joint (TM Joint). Although the exact etiology of rheumatoid arthritis remains unknown, it is suspected that the disease is often initiated by an infectious organism, or by genetic and/or environmental factors. Juvenile rheumatoid arthritis (JRA) is a chronic disease of childhood with a spectrum of joint involvement and associated systemic and other organ involvement. Five percent of all rheumatoid arthritis patients are children. In the United States, approximately 150,000 children are affected by JRA. With upper limb involvement, routine oral hygiene procedures become difficult. Dental evaluations/screenings may not be included in the initial team assessment of these patients until the TM Joint is affected; however, prior to this time, the patient may have had years of poor oral hygiene which could contribute to severe decay and early tooth loss. This case report describes the oral health status of a child with polyarticular juvenile rheumatoid arthritis and the specific recommendations for dental management.  相似文献   

10.
Magnetic resonance imaging (MRI) of the cervical spine was performed on 20 patients (mean age 10 years) with a preliminary diagnosis of juvenile rheumatoid arthritis (JRA). In all patients conventional x-rays of the cervical spine were obtained, and the relationship between clinical status and MRI findings were evaluated. Two patients with clinical manifestations, including neck pain and diminished range of motion, exhibited significant pathologic features on radiogram and MRI, the latter providing more detailed information. Among 18 patients who had no complaints about their cervical spines, 3 patients (65%) had either soft tissue involvement, pannus formation or erosions on the surface of atlantoaxial joints; only four patients (20%) had erosions on plain x-ray views. Since the early diagnostic ability of MRI in JRA allows early therapeutic intervention, every patient with a probable diagnosis of JRA would benefit from MRI.  相似文献   

11.
OBJECTIVE: To evaluate aspects of the natural history of AA amyloidosis complicating juvenile rheumatoid arthritis (JRA), and its response to therapy with chlorambucil. METHODS: Scintigraphy and 7-day turnover studies were performed in JRA patients with histologically proven (n = 35) or clinically suspected (n = 30) AA amyloidosis, following intravenous injection of 123I and 125I-labeled serum amyloid P component (SAP). Prospective monitoring studies were performed over 2-3 years in 20 patients with amyloidosis. All but 2 amyloidosis patients were treated with chlorambucil. RESULTS: Positive scanning results were obtained in all patients in whom imaging was performed within 12 years of positive biopsy findings of amyloid and in 5 patients with clinically suspected amyloidosis. Negative scanning results with normal SAP metabolism, indicating regression of amyloid, were obtained in 4 patients whose amyloidosis had been in full clinical remission for more than 12 years. Prospective monitoring studies in patients whose JRA-associated inflammatory activity was in remission demonstrated regression of amyloid in 8 patients and no substantial changes in 8 others; however, in 4 further patients with active inflammation, there was accumulation of amyloid. There was a very poor correlation between the amount of amyloid present at a particular site and the resultant organ dysfunction. CONCLUSION: Radiolabeled SAP scintigraphy and turnover studies are useful complementary tools in the diagnosis, screening, and quantitative monitoring of type AA amyloidosis in JRA. The amyloid deposits may progress and/or regress at different rates in different anatomic sites over short periods.  相似文献   

12.
OBJECTIVE: To characterize sleep patterns of patients with juvenile rheumatoid arthritis (JRA). METHODS: Sixteen patients with JRA aged 12+/-4 years and 9 controls aged 11+/-3 years underwent a comprehensive evaluation by self-report questionnaire and formal all night polysomnographic recordings. Multiple sleep latency test was performed in 7 patients. RESULTS: Patients had 90% more arousals and awakenings (p<0.01) and the median length of occurrences of uninterrupted sleep in stages 2 and 3 and rapid eye movement (REM) sleep was 60% shorter than in controls (p<0.01). The overall amount of sleep stage shift from deeper to lighter sleep was 23.5+/-10.8 events in patients compared to 14.9+/-4.0 in controls (p<0.05). In 15 of 16 patients 15% of non-REM sleep consisted of alpha-delta (alpha-rating) sleep, compared with less than 1% in controls (p<0.001). Multiple sleep latency test for patients was 10.3+/-2.6 min. There were no differences between JRA and controls in self-reported questions. However, patients reported longer afternoon naps, 1.8+/-1.3 h compared to 0.3+/-0.8 h in controls (p<0.05). CONCLUSION: Objective polysomnographic evidence of abnormal sleep has been confirmed in patients with JRA. Sleep disturbance was associated with daytime sleepiness as evidenced by abnormal multiple sleep latency test and longer afternoon naptime.  相似文献   

13.
OBJECTIVE: To determine in a prospective study the normal MRI morphology of the sacroiliac joints (SIJs) in relation to age and sex during adolescence. DESIGN AND PATIENTS: A total of 98 children (63 boys, mean age 12.7 +/- 2.8 years; 35 girls, mean age 13.7 +/- 2.3 years), ranging in age from 8 to 17 years, with juvenile chronic arthritis (JCA) but without signs of sacroiliitis fulfilled the study prerequisites (no back pain and no pathologic changes of the SIJs on physical examination before MRI in a 1.5-year follow-up). An additional eight HLA-B27-negative boys and eight HLA-B27-negative girls without arthritis served as controls. The MRI protocol comprised a T1-weighted SE sequence, an opposed-phase T2*-weighted GE sequence, and a dynamic contrast-enhanced study in single-section technique. RESULTS: Noncontrast MRI permitted differentiation of "open" from ossified segmental and lateral apophyses of the sacral wings, with a significant difference in age (P < 0.05) between children with open and ossified apophyses. Ossification of the apophyses of the sacral wings was seen significantly earlier (P < 0.05) in girls than in boys. Girls also had a significantly higher incidence of transitional lumbosacral vertebrae, pelvic asymmetries, and accessory joints. In the contrast-enhanced opposed-phase MRI study, normal cartilage of the SIJs showed no contrast enhancement whereas the joint capsule showed a moderate enhancement. CONCLUSION: There are significant age- and sex-related differences in the normal MRI morphology of juvenile SIJs. Our findings might serve as a standard of comparison for the evaluation of pathologic changes--in particular for the early identification of juvenile sacroiliitis.  相似文献   

14.
OBJECTIVE: To determine whether T lymphocyte reactivity to endogenous human hsp60 plays a regulatory role in the course of oligoarticular juvenile rheumatoid arthritis (JRA). METHODS: A prospective, longitudinal study of T cell reactivity to HSP in 15 patients with newly diagnosed HLA-B27 negative oligoarticular JRA was performed. Results were compared with those in a group of 20 patients with newly diagnosed polyarticular or systemic JRA or with acute arthritis caused by other systemic diseases or viral infections, as well as with those in a group of 9 healthy control subjects. RESULTS: In 86% of the patients with oligoarticular JRA (13 of 15), significant T lymphocyte proliferative responses to hsp60 were found in peripheral blood mononuclear cells and/or synovial fluid mononuclear cells within 3 months after the onset of arthritis. Only 5% of the patients in the rheumatologic disease control group (1 of 20) showed such positivity. All patients with oligoarticular JRA and positive responses to human hsp60 developed a remission of their disease within 12 weeks. During this period of remission, blood samples were taken from 8 patients and showed significantly lower and even negative responses to hsp60, compared with active disease, when all 8 patients had good responses. CONCLUSION: The results show that significant proliferative responses to human hsp60 can be found early in the course of oligoarticular JRA. Furthermore, these responses correlate with disease activity in such a manner that T cell reactivity to human hsp60 seems to be associated with disease remission.  相似文献   

15.
OBJECTIVE: To measure serum and synovial fluid (SF) levels of interleukin 8 (IL-8) and monocyte chemoattractant protein-1 (MCP-1) in patients with juvenile rheumatoid arthritis (JRA) and to compare them with adult rheumatoid factor-positive rheumatoid arthritis (RA). METHODS: IL-8 and MCP-1 were measured by immunoassay (1) in sera obtained from 55 children with JRA and from 16 adults with RA, and (2) in SF obtained from 30 children with JRA and 11 adults with RA. RESULTS: Patients with active systemic JRA had serum levels of IL-8 and MCP-1 higher than in controls (p<0.01) and in patients with active polyarticular or pauciarticular JRA (p<0.05). In patients with RA serum MCP-1 levels were higher than in patients with the 3 JRA onset types, while no difference was found for IL-8 levels. Patients with systemic JRA and with current systemic features had serum levels of IL-8 and MCP-1 higher (p = 0.03 and p = 0.04, respectively) than patients in which systemic features had subsided. No significant differences in SF IL-8 or MCP-1 levels were found among the 3 JRA onset types or adults with RA. In patients with JRA SF leukocyte counts were correlated with SF IL-8 levels (p = 0.002), but not with MCP-1 levels. Moreover, SF levels of both IL-8 and MCP-1 were correlated with those of IL-1beta (p<0.001) and IL-6 (p<0.01), but not with those of TNF-alpha. CONCLUSION: Elevated serum levels of IL-8 and MCP-1 in patients with systemic JRA with current systemic features at sampling suggest systemic production of the 2 chemokines during systemic phases of the disease. Similar SF levels of IL-8 and MCP-1 among the 3 JRA onset-types and RA suggest comparable local production of the 2 chemokines.  相似文献   

16.
Intrasynovial injection of corticosteroids is currently one of the routine measures in the treatment of patients with rheumatoid arthritis. Crystalline corticosteroid suspensions have proved their value and are effective for approximately one to three weeks. One or two injections can control synovitis for prolonged periods of time. Special attention has to be given to impeccable technique of instillation. However, it must be kept in mind that, within the overall management of rheumatoid arthritis, the intra-articular injection of corticosteroids is a local, palliative and temporary measure. The intrasynovial instillation of radionuclides is used only in selected cases. Therapy of the knee joint is mainly performed with Yttrium-90, less frequently, the interphalangeal joints are treated with Erbium-169. Costs and radiation exposure are low, but a favourable result is only achieved in slightly more than 50 per cent of cases. This review provides a synopsis of the basic concepts and practical applications of intrasynovial corticosteroid and radioisotope injection therapy.  相似文献   

17.
OBJECTIVE: To characterize Japanese patients having adult Still's disease (ASD) with chronic arthritis (> 6 months) and to examine the association of chronic arthritis with carpo:metacarpal ratio (CMC ratio), an index of radiographic progression in rheumatoid arthritis (RA). METHODS: Twenty-seven patients with ASD (16 women and 11 men, mean age at disease onset 27.7 years) were classified into 2 groups: patients with (chronic articular ASD, 16 patients, 59%) or without (systemic ASD, 11 patients, 41%) chronic arthritis. Clinical and laboratory findings were compared between both groups. CMC ratio was calculated on serial hand radiographs in patients with chronic articular ASD. RESULTS: In our series, serositis was rarely observed in chronic articular ASD. Peripheral arthritis (including transient arthritis), such as metacarpophalangeal, proximal interphalangeal, or ankle joint, was more frequently observed in chronic articular ASD than in systemic ASD (p < 0.05). Wrist arthritis was frequently observed also in systemic ASD; however, joint space narrowing of carpometacarpal or intercarpal joints was recognized only in chronic articular ASD (44%). CMC ratio at the last observation in 14 patients with chronic articular ASD was significantly decreased (0.526 +/- 0.039) compared to that at disease onset (0.553 +/- 0.034) (p < 0.05), while no decrease was observed in 4 with systemic ASD (0.565 +/- 0.062 at disease onset, 0.563 +/- 0.043 at the last observation). CONCLUSION: It is suggested that chronic articular ASD has certain characteristics. CMC ratio may be a quantitative index for assessment of radiographic changes of carpal joints, not only in RA but also in chronic articular ASD.  相似文献   

18.
OBJECTIVE: To assess the long-term health outcomes and quality of life of patients with juvenile rheumatoid arthritis (JRA) using health and functional assessment questionnaires in 2 populations, one from the USA and one from Italy. METHODS: Patient eligibility criteria: (1) first examined in our units between 1958 and 1990 during the first 6 months after onset of symptoms, (2) diagnosis of JRA by the American College of Rheumatology criteria, (3) disease duration of at least 5 years at the time of assessment of outcome. Instruments used: (1) the Health Assessment Questionnaire (HAQ, short form, or childhood HAQ (CHAQ), and (2) Quality of Life Scales (QOLS, adults only). Eligible patients were identified by computer search and chart review and were then mailed a packet containing a consent/assent form and the assessment instruments. RESULTS: Of 346 patients who met the eligibility criteria were able to locate 301, and 290 verbally agreed to participate and were mailed packets. Signed consent and complete information were received from 227 of the 290 (78%), 178 from the USA and 49 from Italy. Mean duration of disease at the time of outcome assessment was 15 yrs. 127 had pauciarticular, 55 polyarticular, and 45 systemic onset disease. Mean and (median) scores of the outcomes are shown in the table. [table: see text] CONCLUSION: Long-term outcome, as assessed by the instruments used, is very favorable in most patients with JRA 5 years or more after onset of symptoms.  相似文献   

19.
To evaluate the effect and tolerance of intraarticular Triamcinolonehexacetonide (TCH) in the course of chronic coxitis in juvenile chronic arthritis (JCA) in an open uncontrolled study. Since 1990 we treated patients < 16 years of age suffering from chronic coxitis with 1 mg/TCH/KG body weight. The patients were checked again 4-8 weeks after the treatment. Clinical and ultrasound courses were recorded with the help of ultrasound and joint scores. The evaluation took place 6, 12, 24 and 36 months after the treatment. At that time we give a report on the 12 months follow-up of 37 hip joints and the 24 months follow-up of 20 hip joints. The immediate effect of TCH influencing mobility, pain sensitivity and joint effusions of the patients is impressing. The long term effect of TCH has to be evaluated by regular check ups for at least 2 years. An individual comparison with the not treated contralateral joint would be desired if ethically justifiable. Most patients suffering from polyarticular diseases with a long course of coxitis needed more than one injection of TCH (mean reinjection time 5.8 months). Any avascular necrosis of the femoral heads or other complications were not observed.  相似文献   

20.
Six children with long-standing juvenile rheumatoid arthritis and radiographic evidence of severe hip joint damage were found 6 months to 5 years afterward to have radiographic evidence of significant hip joint space widening and remodeling of articular surfaces. The disease activity had decreased and hip joint function had improved in all children. Each child had received various antiinflammatory drugs, but no specific medication could be implicated in the improvement. Evidence from animal studies suggests that intensive physical therapy with emphasis on continued weight bearing may have been important in promoting healing in these children. It appears that restoration of severely damaged hip joints in JRA can occur when the disease activity is controlled. Restoration may be favored by a child's greater potential for bone remodeling and by vigorous physical therapy with emphasis upon continued ambulation.  相似文献   

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