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Objective: This study intended to examine the effects of Pilates exercise on pain, cardiorespiratory fitness, functional ability, and quality of life in children with polyarticular juvenile idiopathic arthritis. Methods: Forty children with polyarticular JIA aged 10–14 years old were randomly allocated into two groups: the control group (n = 20) received conventional physical therapy (CPT), and the experimental group (n = 20) received clinical Pilates exercises combined with CPT. Patients in both groups received their program three times/week for 3 months. Pain, cardiorespiratory fitness, functional ability, and quality of life were assessed through the visual analogue scale, cardiopulmonary exercise test, 6 min walk test, and PedsQL scale, respectively, just before and after treatment. Results: Pain (p = 0.001), cardiorespiratory markers (all p < 0.05), functional ability (p = 0.002), and overall quality of life (p = 0.007) improved significantly in the experimental groups compared to the control group. Conclusion: Incorporating Pilates exercises into CPT is likely more effective for decreasing pain intensity, improving cardiorespiratory fitness, augmenting functional ability, and promoting quality of life in children with JIA than CPT alone.

The aim of this study was to assess current evidence for the surgical correction of dentofacial deformities in patients with temporomandibular joint (TMJ) involvement from juvenile idiopathic arthritis (JIA). A systematic literature review, according to the PRISMA guidelines, was conducted. Meta-analyses, randomized controlled trials, cohort studies, observational studies, and case reports were eligible for inclusion. Exclusion criteria were no JIA diagnosis, no clearly defined outcomes, dual publications (except meta-analyses), non peer-reviewed studies, non English language publications, and animal studies. The outcome measures assessed were TMJ function, skeletal alignment, and morbidity. The database search identified 255 citations, of which 28 met the eligibility criteria. Of these, 24 were case reports or case series with a low level of evidence that did not allow for meta-analysis. Extrapolated evidence supports orthognathic surgery in skeletally mature patients with controlled or quiescent JIA and a stable dentofacial deformity. Distraction osteogenesis was recommended for severe deformities. Some authors demonstrated unpredictable postoperative mandibular growth with costochondral grafts. Alloplastic TMJ reconstruction was efficacious, but should be used cautiously in skeletally immature patients. TMJ function and skeletal alignment was improved with reconstruction by any technique and morbidity was low. The surgical correction of arthritis-induced dentofacial deformities is indicated but the level of evidence is low. Prospective multicenter studies are needed.

Temporomandibular joint (TMJ) arthritis is a frequent feature of Juvenile Idiopathic Arthritis (JIA) and affects up to 8 out of 10 children with JIA, irrespective of their JIA subtype [1–3]. For de...

In 1951, Barbara Ansell, a young 28-year-old physician and aspiring cardiologist, was encouraged by Professor Eric Bywaters to join him in the Special Unit for Juvenile Rheumatism, recently created by the Medical Research Council (MRC) of UK, located at the Canadian Red Cross Memorial Hospital in Taplow, Buckinghamshire, UK. She had been advised that cardiology ‘was a man’s world’. Although initially aimed at research and treatment of rheumatic fever, this unit of pathologists and clinicians morphed into a unit for juvenile chronic arthritis as the incidence of rheumatic fever gradually waned. At that time, a child with chronic arthritis would mostly be confined to bed at home, perhaps with aspirin and/or cortisone medication and bore a grim prognosis regarding both function and survival. She began her pioneering work there with her MD thesis (1965) on the classification of children with chronic arthritis. Her clinical skill and experience were legendary. Her major clinical achievements are listed in box 1. She also was actively engaged with scientists in the search for the causes of the diseases she encountered. She was ideally placed in the shared environment of MRC facilities next to a general hospital. Box 2 is a short summary of the main milestones and breadth of her work. In 1988, the world-renowned paediatric rheumatologist, Barbara Ansell CBE, FRCP, FRCS, FRCPCH, retired as director of the MRC Division of Rheumatology at Northwick Park, Harrow, UK. She left as a legacy an army of former colleagues, protegees and friends from all areas of healthcare, dedicated to children and adolescents with rheumatic diseases. By then the outcome for such young patients had improved dramatically. Gratifyingly, Barbara did see the fulfilment of her long-held dream, expressed passionately at her retirement Festschrift: the beginning of truly effective therapies. Box 1 ### Barbara Ansell: Key achievements

Objective.To investigate disease course, outcome, and predictors of outcome in an unselected population-based cohort of individuals diagnosed with juvenile chronic arthritis (JCA) followed for 17 years.Methods.The cohort consisted of 132 incidence JCA cases identified 1984–1986 according to EULAR criteria. At 5-year followup, 129 individuals underwent joint assessment, laboratory measurements, radiographic examination, and medication and functional assessment. At 17-year followup, 86 were examined with joint assessment, laboratory measurements, medication assessment, Health Assessment Questionnaire (HAQ), Keitel functional test (KFT), and Medical Outcomes Study Short Form-36 (SF-36).Results.At 17-year followup, 40% were in remission, 44% changed subgroups, median HAQ score was 0.0 (range 0.0–1.5), and median KFT was 100 (range 54–100). SF-36 scores were significantly lower compared to a reference group. Thirty-nine percent of those in remission at 5-year followup were not in remission at 17-year followup. In multivariate analyses of variables from the 17-year followup: remission was predicted by remission at 5-year followup (OR 4.8); HAQ > 0 by rheumatoid factor (RF)-positivity at 5-year followup (OR 3.6); KFT < 100 by nonremission (OR 11.3); and RF-positivity (OR 5.6) at 5-year followup; and the SF-36 physical component summary score above average of the reference group by remission at 5-year followup (OR 5.8).Conclusion.This longterm study of 86 individuals with JCA showed large variability of disease courses and of impaired health-related quality of life. Sixty percent were not in remission at 17-year followup. Longterm outcome was best predicted by and associated with characteristics at 5-year followup rather than those at onset.

Background The purpose of the study is to determine levels of total cholesterol (TC), low-density, and high-density lipoprotein fractions of cholesterol (LDLc and HDLc), in patients with juvenile idiopathic arthritis (JIA), and relate those to disease activity, overweight, and physical activity (PA), testing the hypothesis that the levels of cholesterol fractions are associated with inflammation as well as with overweight and low PA. Methods Two hundred ten patients with JIA were included in this descriptive cross-sectional study. TC, LDLc, HDLc were measured, and associations with clinical disease activity (JADAS27), biomarkers of inflammation (myelo-related protein complex 8/14 (MRP8/14), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR)), body mass index (BMI), waist-to-height ratio (WtH ratio), and PA were explored. Results Mean values for TC, LDLc, and HDLc in the patients were within the normal range for Danish Children. HDLc was negatively correlated with MRP8/14 (r = −0.343, CI −0.474 to −0.201, p

To investigate, in a population-based cohort of patients with juvenile chronic arthritis (JCA), onset characteristics, progression, outcome, and prognostic factors longitudinally for 5 years.This cohort consisted of 132 incidence cases identified between 1984 and 1986 in southwestern Sweden followed for 5 years with annual reports of subgroup, joint assessment, disease activity, eye examinations, laboratory measurements, and medication. At the 5-year follow-up, the Childhood Health Assessment Questionnaire (Child-HAQ) was evaluated. European League Against Rheumatism (EULAR) criteria for diagnosis and disease activity were used.During the 5 years only four patients were lost to follow-up, 34% changed subgroup and 8% developed uveitis. At the 5-year follow-up the disease was active in 12% of the patients, stable in 28%, inactive in 25%, and in remission in 34%. Among those examined, 24% had radiological changes, of whom half had advanced changes. The Child-HAQ median score at the 5-year follow-up was 0.13 (range 0.0-1.9). The number of involved joints at inclusion correlated positively with active disease at the 5-year follow-up. Age at disease onset, the number of involved joints, and the number of joints with arthritis correlated positively with continuous disease and Child-HAQ score. CONCLUSION. Our study shows a diverse disease course during the first 5 years of JCA where one-third changed subgroup and two-thirds did not reach remission. Age of disease onset, the number of involved joints, and the number of joints with arthritis at inclusion were associated with poor outcome at the 5-year follow-up.

The presence of certain medical or orthopedic conditions need not preclude adolescents from being physically active and participating in sports. The benefits of continued physical activity far outweigh any concerns for potential complications for most such conditions. This article reviews sport participation guidelines for adolescents with conditions that include juvenile chronic arthritis, eye injures, solitary kidney, skin conditions, scoliosis, and spondylolysis.

In 43 patients with pauciarticular and 26 patients with polyarticular juvenile chronic arthritis (JCA), an increased frequency was found for HLA-B27 (36%) in male patients with onset of the disease over 9 years of age. The antigen "DRw6" was observed in high frequency (average 57%) in all subtypes of the disease. Indications for increased frequencies were obtained for DR3 in male JCA (40%) and DR5 (42%) in female JCA patients.

A review of the literature relating to juvenile chronic arthritis (JCA) is presented. It includes a description of the disease itself and identifies our knowledge of the increased risk of dental disease associated with this condition. In addition the impact of JCA therapy on the dental management of the patient is discussed. The review highlights the need for further research into the effects of the disease on dental caries, periodontal disease and saliva.

Nutritional status and nutrient intake were assessed in 17 children with active juvenile chronic arthritis (JCA) who never received steroids and in 17 controls matched for age and sex. Five patients had systemic, seven polyarticular and five oligoarticular JCA. Values significantly below those of the controls were found in systemic patients for height (p < 0.05), upper arm circumference (p < 0.05) and arm muscle area (p < 0.01), and in polyarticular subjects for arm muscle area (p < 0.01). All patients had unremarkable anthropometric fat measurements. All anthropometric measurements were normal in oligoarticular patients. Twelve JCA patients had reduced serum iron (Fe), 6 reduced serum zinc (SZn), 14 reduced intra-erythrocytic zinc (EZn) and 2 reduced serum copper (SCu). SZn was inversely correlated with erythrocyte sedimentation rate (ESR) (p= 0.023). EZn was inversely related to lymphocyte count (p= 0.022). SCu was related to ESR (p= 0.037) and to lymphocyte count (p= 0.016). No significant difference in nutrient intake was found between patients and controls. Active JCA was associated with reduced muscular mass, Fe, SZn and EZn. These alterations did not depend on reduced nutrient intake. Juvenile chronic arthritis, nutrient intake, nutritional status, trace elements

Juxtaarticular osteoid osteoma is frequently misdiagnosed because the symptoms may mimic arthritis, and radiographs may not be characteristic. A rare case of subtalar pain lasting 5 years in a female teenager is presented here. The initial diagnosis was monarticular juvenile chronic arthritis. Family history was misleading because her mother had rheumatoid arthritis (RA). Arthritis Rheum

We retrospectively assessed linear growth and final height in a group of 24 patients suffering from juvenile idiopathic arthritis (JIA) during childhood, receiving steroid therapy. In these patients, a significant loss of height (–2.7 ± 1.5 SDS) occurred in the first years of the disease which was positively correlated with prednisone therapy duration. After remission of the disease and prednisone discontinuation, most of the patients (70%) had catch-up growth but 30% had a persistent loss of height. Their mean final height was strongly correlated with their mean height at the end of steroid therapy and was significantly different between the group of patients with catch-up growth (–1.5 ± 1.6 SDS) and the group without catch-up growth (–3.6 ± 1.2 SDS). This pattern of growth observed in JIA patients should help us to define strategies of GH treatment in these patients in order to improve their final height. We have previously reported the beneficial effects on growth and body composition of a 1-year GH treatment in a group of 14 growth-retarded patients suffering from juvenile idiopathic arthritis, receiving glucocorticoid therapy. These patients (n = 13) were treated again with GH at the same dosage (0.46 mg/kg/week) for another 3-year period. GH treatment markedly increased growth velocity in these patients, but had a minor effect on SDS height suggesting that these children will remain short at adult age. Using GH earlier in these patients during the course of their disease may prevent growth deterioration and metabolic complications induced by chronic inflammation and long-term steroid therapy.

Objectives: We sought to study the long-term outcome of juvenile chronic arthritis (JCA) in the temporomandibular joint (TMJ). Study Design: Temporomandibular disorders, including TMJ involvement, were assessed in 42 women with pauciarticular or polyarticular JCA—on average 25.8 years from disease onset—and compared with those found in matched control subjects. Disease-related parameters associated with temporomandibular disorders were identified. Results: The TMJ was involved in 66.7% of the patients, most severely in extended pauciarticular JCA. Temporomandibular disorders were more frequent in the patients than in the control subjects, especially in those with persistent disease. The TMJ involvement was positively correlated with disease duration and negatively correlated with jaw opening and occlusal support. Duration of active JCA and history of functional pain were identified as predictors of present TMJ involvement. Conclusion: In a long-term follow-up, TMJ involvement proved frequent in the studied patients and was associated with long disease duration and previous pain on jaw opening. The findings suggest that patients with JCA should undergo orofacial evaluation on a regular basis. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92:406-14)

The purpose of our study was to find out the intensity of free radical reactions in pediatric patients with different forms of juvenile chronic arthritis (JCA) on the basis of carbonyl groups’ content in plasma proteins, evaluated with the use of Levine’s method. We examined a population of 52 children with diagnosed JCA of different types and activities in the study. The carbonyl groups’ content in plasma proteins of ill children was significantly higher than in healthy group (1.36 ± 0.68 vs. 0.807 ± 0.16 nmol/mg of protein). The carbonyls increased parallell with the activity of the disease; their content was significantly higher in children with high-disease activity than in children with medium- or low-disease activity. Moreover, children with oligoarthritis had carbonyls level 1.09 ± 0.59 nmol/mg of protein, vs. 1.62 ± 0.82 in children with systemic type of JCA. The values of carbonyls in children with polyarthritis were and 1.36 ± 0.50 nmol/mg of protein. Correlation between the carbonyl groups content and the activity or the type of JCA may allow use of carbonyls as a clinical marker of antioxidant barrier impairment in this group of patients. This aspect of the disease may undergo pharmacologic modification in future.

We present the results of antineutrophil cytoplasmic antibody (ANCA) staining in patients with juvenile chronic arthritis (JCA). Thirty-one patients with an age range of 1-16 years were included in the study: 13, 15 and three patients, respectively, were classified having oligoarticular, polyarticular and systemic-onset disease. Indirect immunofluorescence analysis revealed ANCA staining in 45% of the patients. All, except one, revealed atypical pANCA staining. ELISA studies for anti-myeloperoxidase were positive in only one patient with typical pANCA staining. PR-3 ANCA tested negative in all patients. There were no significant correlations between ANCA staining and the clinical parameters of the patients. We conclude that, although the specificity of ANCA in JCA remains to be elucidated, it may be effective in the pathogenesis of the disease.

A 25-year-old woman with recurrent pronounced enlargement of the iliopsoas bursae is described; her hips were evaluated by ultrasonography and MRI. They appeared normal and communications to the iliopsoas bursae were demonstrated. During the preceding 8 years she had intermittent seronegative non-erosive arthritis of the knee and finger joints and her disease was classified as juvenile chronic arthritis.

A total of 371 serial dental panoramic radiographs from 71 children with juvenile chronic arthritis (JCA) were examined to determine the presence and extent of radiographically detectable condylar abnormalities. The series included 12 children with so called ‘bird face’ deformity and 55 in whom facial growth was judged to be normal. By the age of 15 years, 27 patents (38 per cent), showed lesions of the TMJ, of which 14 (55 per cent) were bilateral. Of those cases with unilateral destruction, the left condyle was affected nine times more frequently than the right. All of the children with ‘bird face’ deformity had condylar abnormality, but these facial characteristics should not be condidered pathognomomic of juventile chronic arthritis. Moderate to severe condylar abnormality was detectable in 10·9 per cent of children with normal facial growth, and where condylar destruction is present it can often be established as early as 8 years. Systemic corticosteroids appear to have little or no effect on the condyle or mandibular growth.

The intestinal microflora was indirectly evaluated in juvenile chronic arthritis (JCA) by analysing enzyme activities--urease, beta-glucosidase and beta-glucuronidase--in faeces. In 18 out of 26 JCA patients, the illness had been diagnosed during the past year. The control group was composed of eight age-matched control patients and 18 family members of JCA patients (3-36 yr). The mean [95% confidence interval (CI)] urease activity, but not the activities of beta-glucosidase and beta-glucuronidase, in faeces from the JCA group differed from that in the control group: 32.3 (26.6-38.1) nmol/min/mg protein vs 24.0 (16.8-31.6), P = 0.07. The difference was more marked in a comparison of JCA patients with family members (P = 0.03). In a subgroup of subjects, the effect of 10 days oral bacteriotherapy with Lactobacillus GG on faecal enzyme activities was then investigated (n = 8 JCA patients, n = 8 control patients). This short-term oral bacteriotherapy reduced the increased urease activity in faeces of JCA patients. Keeping in mind the small number of subjects, it may be inferred from the present results that the increased urease activity in JCA is specific for the disease, suggesting altered intestinal microflora in JCA.

In juvenile chronic arthritis (JCA) the indices of disease activity, such as joint swelling, pain, tenderness, and limitation of motion, may poorly correlate with the degree of functional impairment. Since the improvement of functional capacity is one of the main objectives of treatment, we evaluated the effect of methotrexate (MTX) in 29 children with JCA by assessing both the articular indices and a functional status measure. After 6 months of therapy, 15 of the 29 patients had a significant (or = 50%) reduction in the number of joints with active arthritis and/or the articular severity score and were then judged as responders, while 14 did not respond to MTX. In the responder group, the median functional index score decreased from 17 (range: 13-27) at baseline to 13 (range: 13-15) at 6 months (p0.001); the median score of the non-responder group was 20 both at baseline and at 6 months (ranges: 14-27, and 13-36, respectively). These results show an improvement of functional impairment in those patients who respond to MTX according to the conventional indices of articular inflammation.

Summary That juvenile chronic arthritis (JCA) occurs in tropical countries there is no doubt. The spectrum of differential diagnoses is wider than in temperate regions. The disease tends to be more severe irrespective of the type of onset, and there is a high incidence of positive rheumatoid factor. Many cases present with advanced disease. There is a need for the establishment of clinics devoted specifically to the detection, documentation, and treatment of JCA.

Chronic iridocyclitis (CI) is among the most important extra-articular manifestations of juvenile chronic arthritis (JCA) and is most often observed in young girls with pauciarticular JCA and circulating antinuclear antibodies (ANAs). The frequency of CI found in 39 children with ANA-positive pauciarticular JCA was 56%. None of the patients developed ocular complications during follow-up. We think that this favourable outcome was primarily due to the early diagnosis and to our choice of using atropine as a mydriatic agent and to prolong atropine, after having spaced its administration further apart, up to one year or longer after the remission of ocular manifestations.

Twenty-nine children with juvenile chronic arthritis, aged from 7 to 16 yr were studied using the Juvenile Arthritis Functional Assessment Report for Children and Parents questionnaires (JAFAR-C and JAFAR-P). The results showed that JAFAR-C and JAFAR-P were highly correlated (P < 0.001) with each other and also with a predicted JAFAR score (P < 0.05). The JAFAR score correlated with the arthritic joint count at the time of the study (P < 0.01), Steinbrocker classification (P < 0.001), pain score (P < 0.01) and stiffness score (P < 0.005). Evidence of psychological dysfunction was found in eight of the 29 children (27%). Six children (21%) scored 13 or more on the Rutter A(2) for parents and one patient scored more than nine on the Rutter B(2) for teachers. Only one child had a score on the Birleson self-rating scale that was high enough to suggest a likely diagnosis of depression. The median Lipsitt self-concept scale score was 86, that expected for a healthy population. There was no correlation between the psychological scores and any of the other functional measurements, although the numbers affected were small. There was no significant difference between type of arthritis at onset with regard to any of the scores obtained.

A comprehensive assessment of 10 adolescents (mean age 15.7 years) fulfilling the ACR criteria for fibromyalgia, disclosed that 3 patients also had juvenile chronic arthritis. Based on semi-structured psychiatric interviews, testing and family assessments, 6 of the patients had a psychiatric diagnosis (over anxious and/or depressive disorders). The pain scores for the group (mean 5.0, SD 1.5) were significantly higher than for a comparison group of patients with juvenile chronic arthritis (mean 2.5, SD 1.7), (p < 0.01). Average IQ was normal (mean 102.3, SD 13.9), but striving for achievement and high parental expectations were evident in 8 families. Seven of the mothers and 3 of the fathers had chronic diseases. The frequency of individual and family stress indicates a need for psychosocial assessment and counselling soon after onset of symptoms. This study also serves as a reminder that the diagnosis of juvenile chronic arthritis does not exclude fibromyalgia.

Sulphasalazine in a dose of 50 mg/kg/day was administered to ten patients with pauciarticular-onset juvenile chronic arthritis (JCA), with active disease not adequately controlled by nonsteroidal anti-inflammatory drugs (NSAID). The treatment was initiated with 1/4 of this dose and increased by weekly increments of 250-500 mg until the total dose was reached. In all patients sulphasalazine was the first disease-modifying agent tried. Among nine of the ten patients there was significant improvement in all clinical scores, including the number of active joints and the severity grading (tenderness and limitation of motion). Within 3 months of sulphasalazine therapy the laboratory measurements revealed marked improvement in the erythrocyte sedimentation rate (ESR) and haemoglobin values. One patient, in whom the ESR and haemoglobin were normal at onset, had no change in clinical scores. Transient skin rash and elevated liver enzyme levels developed in one patient. These preliminary data suggest that sulphasalazine is an effective and safe second-line agent in the management of pauciarticular-onset JCA. More trials with this drug are needed, including double blind, to study efficacy and safety of sulphasalazine in JCA.

Nutrient intake and nutritional status were assessed in 15 children with juvenile chronic arthritis (JCA) and in 17 healthy controls. Anthropometric measurements were similar in children with pauciarticular JCA and in controls, whereas weight (p = 0.05) and upper arm muscle area (UAMA) (p less than 0.01) were reduced in children with polyarticular JCA. Compared with healthy controls the concentrations of hemoglobin, serum iron and serum zinc were reduced in the children with polyarticular JCA (p less than 0.01) and serum copper was increased (p less than 0.01). In the patients the concentrations of hemoglobin, serum iron and serum zinc correlated negatively with erythrocyte sedimentation rate (ESR), whereas serum copper correlated positively. Impaired nutritional status was found in the children with polyarticular JCA in spite of increased energy and protein intake. In this group of patients the dietary intake of calcium was also found to be reduced.

We describe a patient with juvenile chronic arthritis who developed reactive angioimmunoblastic lymphadenopathy, induced by sulfasalazine. Development of angioimmunoblastic lymphadenopathy although rare, is a very serious side effect of sulfasalazine treatment, and patients treated with this drug should be watched carefully.

We review the results of 96 primary total hip replacements in 54 patients with juvenile chronic arthritis at five years or longer after surgery. The mean age at operation was 16.7 years (range 11.25 to 26.6); the follow-up period averaged 11.5 years. The clinical results in terms of pain, range of movement, mobility and function are presented. A revision procedure was required in 24 hips (25%) in 18 patients at an average of 9.5 years after the primary operation. A further 17 hips had radiographic signs of loosening. The factors thought to contribute to this relatively high failure rate in patients with juvenile chronic arthritis are discussed.

Insulin-like growth factor (IGF-1) concentrations were determined in a series of 23 children with juvenile chronic arthritis in conjunction with anthropometric assessment. When standardised z scores were used significant decreases in height and weight were shown in comparison with the normal age/sex matched means. Severe growth disturbance was seen, particularly in those with prolonged disease duration, which was independent of corticosteroid treatment, indicating disease activity itself is a major factor in the growth retardation. Eight children had low IGF-1 z scores--that is, less than -2.00 from age/sex matched mean. Low IGF-1 z scores were associated with low weight z scores but not with low height z scores. Overnight growth hormone secretory profiles were determined in 10 patients, including seven with low IGF-1, and showed generally normal secretion in all but one patient, who subsequently attained normal concentrations coincident with catch up growth. Increased pulse frequency of overnight secretion was commonly seen. Low IGF-1 concentrations probably result from varying factors, particularly nutritional, but do not reflect marked endocrinological abnormalities in most patients.

Pirprofen (100 or 200 mg; Rengasil) was administered to experimental groups of children (children with juvenile chronic arthritis, JCA) and to a control group of children (children without JCA) as a single dose or as repeated doses. The pharmacokinetics of pirprofen in these children were compared to the pharmacokinetic parameter values obtained in healthy volunteers and in elderly arthritic adults receiving 400 mg of pirprofen. The children were examined regularly and laboratory values were determined in order to detect possible side effects. The results demonstrated that the pharmacokinetics of pirprofen were similar for children and adults when taking into account the dose and the body weight. There was no drug accumulation after repeated administration of pirprofen. As already observed in rheumatic adults, pirprofen remains in synovial fluid longer than in plasma.

IntroductionJuvenile idiopathic arthritis (JIA), formerly known as juvenile rheumatoid arthritis (JRA) in the United States and referred to as juvenile chronic arthritis (JCA) in Europe, is a group of idiopathic arthritides with an onset before 16 years of age that persists for at least 6 weeks.1,2

Background: Juvenile chronic arthritis (JCA) is a chronic arthritis affecting children below age of 16 years. The systemic onset subgroup is also known as Still's disease. There are several distinct subgroups. There is paucity of literature of this disease entity in our environment due to under diagnosis of the disease. Method: The case note of this patient was retrieved. He was managed for 2 years in the adult rheumatology clinic after being transferred from the Paediatric unit. Relevant literature was reviewed. Result: There was a good response to immunosuppressive agents and low dose prednisolone. He was back at School and able to play with his mates after long withdrawal from School. Conclusion: Prompt referral of such cases to specialist centers will go a long way in determining the outcome of such patients. Prognosis is better with early presentation and appropriate management. Nigerian Journal of Medicine Vol. 17 (1) 2008 pp. 112-114

Over a 22-year period, eight patients affected with severe systemic or polyarticular juvenile chronic arthritis (JCA) developed systemic amyloidosis with nephrotic syndrome. They were treated with chlorambucil over 5-192 months (mean = 44 months). With treatment, an abrupt decrease in the severity of JCA was observed in six patients but two patients were chlorambucil resistant. After a mean follow-up period of 10 years from onset of renal symptoms, one chlorambucil-resistant patient died of end-stage renal failure; two patients have a persistent nephrotic syndrome; and five patients are free from proteinuria, of whom one has developed hypertension. A good correlation was observed between the response of the rheumatic disease to chlorambucil treatment and the clinical course of renal symptoms. Fourteen renal biopsies were performed in these eight patients. In all, amyloid deposits were of the AA type, which persisted on repeat biopsies. In addition, 15%-60% of glomeruli had become globally sclerotic by the second or third biopsies. At the ultrastructural level, modifications in the structure of amyloid deposits and reparative changes of the glomeruli, characterized by partial restoration of glomerular architecture, were observed in three patients with a favourable clinical course.

At their first admission to hospital, 57 children (7-16 yrs) with definite or suspected rheumatic disease were interviewed regarding their perceptions of quality and intensity of pain and disease severity. All children reported pain. This was most often described as 'aching' (74%). The assessments of quality and quantity of pain by the children did not differ between diagnostic, age, or gender groups, or between children with or without previous exposure to other persons with rheumatic diseases. Actual pain intensity was correlated with disease severity assessed by the child and the parent. However, pain intensity was not correlated with a global assessment of disease severity by the physician or the child's psychosocial functioning. These findings may indicate a need for more generally accepted criteria of disease severity and pain assessment in patients with JCA.

Antihistone antibodies were detected in the sera of a randomly selected group of patients with rheumatoid arthritis (RA) and juvenile chronic arthritis (JCA) by an enzyme-linked immunosorbent assay (ELISA) with the five individual histones and by immunoblotting. In ELISA, the overall frequency of antihistone antibodies in RA and JCA was 51 and 44%, respectively. Antibodies present in these serum samples were further studied by ELISA by means of 17 core histone synthetic peptides. The fragment 1–21 of H3 was recognized by 60% of RA sera and by 62% of JCA sera. In addition, at least four terminal or internal peptides of H3 and H4 were recognized by more than a third of JCA sera, while only two of these peptides reacted with 20% of RA sera. Many of the sera that did not show any reactivity with the whole histone reacted with various histone peptides. This finding demonstrates the usefulness of synthetic peptides for identifying autoantibodies.