Form Of Juvenile Chronic Arthritis Research Articles

THERAPY OF R EFRACTOR Y JUVENILE CHRONIC ARTHRITIS

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Protein carbonyl groups’ content as a useful clinical marker of antioxidant barrier impairment in plasma of children with juvenile chronic arthritis

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.

Autologous haemopoietic stem-cell transplantation in four patients with refractory juvenile chronic arthritis

Autologous haemopoietic stem-cell transplantation (AHSCT) had been described as a possible treatment for severe autoimmune disease refractory to conventional treatment. We report the first four children with severe forms of juvenile chronic arthritis (JCA) treated with AHSCT. We studied three children with systemic JCA and one child with polyarticular JCA. Unprimed bone marrow was taken 1 month before AHSCT. T-cell depletion of the graft was done with CD2 and CD3 antibodies. We used a preparative regimen of antithymocyte globulin (20 mg/kg), cyclophosphamide (200 mg/kg) and low-dose total body irradiation (4 Gy). Methotrexate and cyclosporin were stopped before AHCST, prednisone was tapered after 2 months. Our patients showed a drug-free follow-up of 6-18 months with a marked decrease in joint swelling, pain, and morning stiffness. Erythrocyte sedimentation rate, C-reactive protein, and haemoglobin returned to almost normal values within 6 weeks. Despite T-cell depletion there was a rapid immune reconstitution in three out of four children. Two patients developed a limited varicella zoster virus eruption, which was treated by aciclovir. AHSCT for severe JCA was well tolerated and induced a remission of disease in four children with JCA that was resistant to conventional treatment. Prolonged prednisone-free growth catch-up and general well-being is a major therapeutic gain in such children. The actual follow-up is too short, however, for us to conclude that these children are completely cured of their disease.

Beneficial effects of one-year growth hormone administration to children with juvenile chronic arthritis on chronic steroid therapy. I. Effects on growth velocity and body composition.

Severe growth retardation and profoundly altered body composition are observed in children with systemic forms of juvenile chronic arthritis receiving glucocorticoids. The purpose of this study was to assess the effects of recombinant human GH (rhGH) on growth velocity (GV) and body composition studied by dual-energy X-ray absorptiometry, during a 1-yr treatment course, together with potential adverse effects on glucose tolerance. Fourteen patients were treated with rhGH (1.4 U/kg per week) for 1 yr and were then studied for a 2nd yr off GH. Baseline GH secretion, GH binding protein (BP), insulin-like growth factor-I (IGF-I), and IGFBP3 levels were at the lower limit of normal. The rhGH treatment increased IGF-I and IGFBP3 plasma levels to above-normal values. All patients showed an increase in GV, and mean GV increased from 1.9-5.4 cm/yr (P < 0.001). Compared with the value on day 0, lean body mass increased by 12.2% (P < 0.01), and the fat mass excess fell by 19.5% (P < 0.01). Decreased glucose tolerance (as determined by oral glucose tolerance test) and increased glycosylated hemoglobin levels were observed during treatment. This effect may be attributed to insulin resistance, as reflected by induced hyperinsulinemia. Eleven children were monitored for 1 yr after the cessation of GH therapy. GV fell to pretreatment values, whereas height in SD score at the end of the 2nd yr was lower (P < 0.01) than before treatment. Weight and fat mass again increased markedly. Although long-term controlled studies are needed to assess the risks and benefits of GH therapy in this setting, our results suggest that rhGH may partially counteract the adverse effects of glucocorticoids on growth and metabolism in patients with chronic inflammatory disease.

The effect of methotrexate and azathioprine on the serum levels of IgA-alpha 1-antitrypsin complex in juvenile chronic arthritis.

In the present study we investigated the influence of methotrexate (MTX) and azathioprine (AZA) on the serum levels of the IgA-alpha 1-antitrypsin (IgA-AT) complex in patients with the systemic form of juvenile chronic arthritis (JCA). Fifty-six JCA patients (22 treated with MTX, 18 treated with AZA, and 16 not treated with any immunosuppressive agent) were enrolled in the study. MTX dosage ranged from 0.3 to 0.5 mg/kg-1 week-1, while AZA was given daily at an average dose of 1 mg/kg. MTX was given for 13 months (SD = 7 months) whereas AZA for 11 months (SD = 6 months). The average value of the complex was higher in JCA patients than in both control groups (0.74 +/- 0.73 U vs 0.37 +/- 0.13 U (control children), P < 0.001 and vs 0.23 +/- 0.12 U (control adults), P < 0.001). Values exceeding the normal range were found in twenty-two JCA patients (39.4%). Serum IgA-AT level was lowest in the MTX group compared to AZA and non-treated patients (0.56 +/- 0.24 U, 0.76 = 0.43 U, 0.95 +/- 0.52 U, respectively, P < 0.05). IgA values exceeding normal levels for age were found in 14% of the patients. A correlation between the levels of the IgA-AT complex and C-reactive protein (r = 0.43, P < 0.01), alpha 1-acid-glycoprotein (r = 0.45, P < 0.01), alpha 1-antichymotrypsin (r = 0.52, P < 0.01), alpha 1-antitrypsin (r = 0.40, P < 0.01) and IgA (r = 0.56, P < 0.01) was established.

Antiperinuclear factor in the polyarticular form of juvenile chronic arthritis: are use of frozen material or age of patients relevant?

Antiperinuclear factor in the polyarticular form of juvenile chronic arthritis: are use of frozen material or age of patients relevant?

Childhood arthritis: Still’s disease

Jan Maycock continues our occasional series on uncommon disorders by describing the features and management of the acute systemic form of juvenile chronic arthritis in children

Genetic Aspects of Juvenile Chronic Arthritis

Immunogenetic studies in the past decade have confirmed the theory of an association between one's immunogenetic background and the manifestation of several forms of juvenile chronic arthritis (JCA), in particular pauciarticular-onset JCA. Considerable work has been done at the serologic level to demonstrate disease association with the major histocompatibility Class II antigens in pauciarticular-onset JCA. The polygenic nature of JCA is best illustrated by the findings in this particularly well-defined clinical subgroup. Evidence is now emerging that analysis of the DNA sequence, the derived protein sequence, and the structure of Class II molecules will yield significant insight into the genetic predisposition to JCA.

Die Dipeptidylpeptidase-IV-Aktivität im Serum und in der Synovia bei Patienten mit juveniler chronischer Arthritis (jcA)

The activity of dipeptidyl-peptidase IV (EC No. 3.4.14) in the serum is measured on 50 healthy children and adolescents and a normal range of 687 ± 222 nmol/l . s is determined. Measurement of the enzyme concentration in the serum was done on 100 patients with juvenile chronic arthritis and in 48 of these also in the synovia. In the serum, the DPIV activity decreases with increasing serological inflammatory signs. There is no connection between this and the clinical form of juvenile chronic arthritis or the therapeutic regimen. DPIV activity in the synovia is always lower than in the sera of the same patients. With increasing inflammatory activity in the joint there is a trend to decreasing concentration of the enzyme in the synovia.

Immunogenetics of Juvenile Chronic Arthritis

The current knowledge of the relationship between the HLA system and the different forms of Juvenile Chronic Arthritis is reviewed: Of the different forms the Early Onset Pauciarticular JCA is associated with DRw8, DR5 and A2, the Polyarticular Onset Rheumatoid Factor positive JCA shows the same pattern of association as does the Adult Rheumatoid Arthritis namely with DR4 and DR1. Juvenile Spondylitis is strongly related to the presence of B27. For the other clinical forms of Juvenile Chronic arthritis the data are not sufficiently well established to give a definite association. In Early Onset Pauciarticular JCA the association with the DR antigens DRw8 and DR5 is independent of that with HLA-A2. Thus there must be at least two HLA linked regions contributing to susceptibility to this form of JCA, one in the region of HLA-A and the other one in the HLA-DR region. If Early Onset Pauciarticular JCA is analysed according to the type of onset (Monoarticular, with large joints, with large and small joints, with small joints only and with Iridocyclitis) there are no significant differences in the frequencies for the associated DR alleles DR5 and DRw8. The antigen DR4 is not found at all in patients with Monoarticular Onset and in increasing frequencies with the increased number of involved joints. Analysis of the same patients according to the clinical course of the disease (Persistent Pauciarticular, Extended Pauciarticular, Polyarticular) shows that again there are no significant deviations for the associated antigens A2, DR5, DRw8. DR4 is not found in the group with Persistent Pauciarticular Disease.

Gold compounds in rheumatic diseases--1.

Gold complexes diminish synovitis and improve therapeutic outcome in rheumatoid arthritis, psoriatic arthritis, some forms of juvenile chronic arthritis, and palindromic rheumatism. The decision to treat a patient with a gold compound should not be undertaken lightly. It should be understood by the patient that the commitment to therapy is potentially long-term, that major benefit is not assured, and that there is, approximately, a 50% probability of an adverse reaction which may terminate treatment. Nevertheless, most adverse effects are mild and transient and the probability of good control, even remission, in an otherwise threatening disease, is sufficiently high to justify a therapeutic trial in the majority of patients. Those patients whose rheumatoid arthritis appears to respond to gold may be encouraged to continue long-term therapy with maintenance doses, in view of the evidence of sustained efficacy in most patients and of the declining risk of adverse reactions after the first 12 months.

Severe aortic insufficiency in juvenile chronic arthritis

Valvular heart disease is rare in patients with juvenile chronic arthritis. We describe a 27-year-old woman with the systemic-onset form of juvenile chronic arthritis in whom aortic insufficiency necessitated valve replacement. Nodules were seen on both the aortic and anterior mitral leaflets at surgery, and histopathologic evaluation of the excised aortic leaflets demonstrated nonspecific changes similar to those described in rheumatoid valve disease causing aortic insufficiency in adults with rheumatoid arthritis. We believe that this is the first reported case of aortic insufficiency in systemic-onset juvenile chronic arthritis in which the pathologic condition of the valve can be attributed to the underlying disease.

Antigen-antibody complexes in the serum of patients with juvenile chronic arthritis.

IgG-containing antigen-antibody complexes were detected in the sera of all of 7 patients with the systemic form of juvenile chronic arthritis, usually at high levels. Only 9 of 25 patients with the polyarticular form had such complexes, usually at low levels. Three patients had low levels of serum IgA which were probably drug induced; the one patient with low C2 and the 3 with low yeast opsonisation were probably not more than to be expected in a random population.