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Biomedical subjects

A Ravelli

Publications and source records attributed to A Ravelli.

At least 127 records · Page 7Linked to original sources
PubMedDate not supplied

Combination therapy with methotrexate and cyclosporine A in juvenile idiopathic arthritis.

OBJECTIVE: To investigate the efficacy and safety of a combination therapy with methotrexate (MTX) and cyclosporine A (CyA) in patients with juvenile idiopathic arthritis (JIA) who were refractory to MTX as a single second-line agent. METHODS: Seventeen consecutive patients with JIA who had refractory polyarthritis despite a minimum of 6 months of MTX as a single second-line agent at the dose of 15 to 25 mg/m2/week were continued with MTX with the addition of CyA (4 mg/kg/day) for 6 to 30 months (median 10 months) were analyzed. The clinical response to therapy was assessed through the preliminary definition of improvement in JIA. RESULTS: At the end of the treatment, as compared to the time when CyA was added to MTX, 8 patients (47%) met the 30% definition of improvement; among them 5 (29%) met the 70% definition of improvement, and 2 (12%) achieved complete disease control. Seven patients (41%) experienced side effects: 4 gastrointestinal discomfort, 1 liver transaminase elevation, and 2 increase > or = 30% in the serum creatinine concentration. No patients was discontinued from combination therapy due to adverse events. CONCLUSION: In our JIA patients who were refractory to MTX as a single second-line agent, the addition of CyA was associated with a significant clinical improvement in roughly half of the patients.

Administration, Oral↗
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Early predictors of outcome in juvenile idiopathic arthritis.

The definition and management of "early arthritis" in children differ from those in adults because juvenile idiopathic arthritis (JIA) is markedly different from adult rheumatoid arthritis. Since a significant proportion of patients with JIA develop articular damage and enter adult life with persistently active disease, it is important to predict early in the disease course the long-term outcome in order to tailor treatment to the risk of disability. Over the past 3 decades a number of studies have evaluated the long-term outcome of cohorts of patients with JIA and some of them have also attempted to identify early prognostic factors. In summary, greater severity/extension of arthritis at onset, symmetric disease, precocious hip/wrist involvement, the presence of rheumatoid factor, and prolonged active disease were the best predictors of a poor outcome. Specific correlates for systemic JIA were persistent systemic features and thrombocytosis at 6 months following presentation, whereas joint symmetry and a higher erythrocyte sedimentation rate at onset were associated with a more severe course in oligoarticular JIA. However, although data is accumulating on prognostic factors in JIA, prediction of long-term outcome in the first few months remains difficult. To better define prognostic factors in future analyses, a considerable effort should be made to increase standardization among studies. Furthermore, a radiographic scoring system and a set of remission criteria specific for JIA should be developed.

Adolescent↗
PubMedDate not supplied

Toward an understanding of the long-term outcome of juvenile idiopathic arthritis.

Over the past four decades, a number of studies have evaluated the long-term outcome of juvenile idiopathic arthritis (JIA) and some of them have also attempted to identify early prognostic factors. This editorial addresses, by reviewing the surveys that have analyzed the outcome of JIA in term of clinical remission, physical disability, and radiographic damage, the clinical questions that are most relevant in this area of study. Altogether, the available data indicate that JIA is not a benign disease because a considerable number of patients still enter adulthood with persistently active disease and a significant proportion of them may develop severe physical disability. Among the different onset forms, the long-term outcome is best in persistent oligoarthritis and worst in RF-positive polyarthritis; the outcome of systemic arthritis is widely variable, perhaps reflecting the heterogeneity of this JIA subtype. The comparison of earlier studies with those published in the last decade shows a decline in the frequency of patients with severe physical disability over the years; however, the proportion of patients who enter adulthood with active disease does not seem to be diminished. Although there is considerable data on prognostic factors in JIA, prediction of long-term outcome early after disease presentation is still difficult because comparisons among studies are hindered for a variety of reasons. Thus, while a considerable body of data is accumulating, the definition of the long-term outcome of JIA remains imperfect. To increase the comparability of future analyses and to obtain generalizable information on the prognosis of JIA and its prediction, a great deal of effort should be directed toward standardizing the study design and the measurement of predictors and outcomes.

Arthritis, Juvenile↗
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Functional ability and physical and psychosocial well-being of hypermobile schoolchildren.

OBJECTIVE: To compare the functional ability and the physical and psychosocial well-being of children with joint hypermobility to those of age- and sex-matched non-hypermobile subjects. METHODS: 311 healthy Italian schoolchildren aged 6.3 to 19.3 years were examined for hypermobility of the joints. Functional ability was assessed through the Childhood Health Assessment Questionnaire (CHAQ) and the physical and psychosocial well-being through the Childhood Health Questionnaire (CHQ). The parent's assessment of the child's overall well-being and of the child's pain was measured on a visual analogue scale. RESULTS: The overall prevalence of articular hypermobility was 34% (106/311), with the median hypermobility score being 3 (interquartile range 1, 5). Although the hypermobility score of girls (median 3: interquartile range 2, 5) exceeded that of boys (median 2.5; interquartile range 0, 5), this difference was not statistically significant (p = 0.16). The level of hypermobile children's pain in the preceding weeks, as assessed by the parents, was comparable to that recorded in the non-hypermobile peers. There was a weak negative correlation between the hypermobility score and the age of the child (r = -0.14, p=0.01). All instrument scores were comparable between hypermobile and non-hypermobile subjects, with the sole exception of a borderline significant greater impairment of the Role/social limitations-physical subscale of the CHQ in the hypermobile group. The hypermobility score was not correlated with any instrument score. CONCLUSIONS: The presence of joint hypermobility does not affect the functional ability and the physical and psychosocial well being of otherwise healthy children. These results suggest that the physical functioning in everyday life and the general health status of hypermobile children are not impaired.

Adolescent↗
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Unilateral destructive wrist synovitis in juvenile idiopathic arthritis.

OBJECTIVE: To describe the clinical and radiographic features of a group of juvenile idiopathic arthritis (JIA) patients who developed unilateral destructive wrist synovitis. METHODS: All wrist radiographs performed yearly between 1986 and 2002 in JIA patients who had wrist involvement were retrospectively reviewed to identify patients who had unilateral erosive wrist synovitis, defined as a difference of at least -3 units in the Poznanski score between the affected wrist and the unaffected wrist, with the Poznanski score in the unaffected wrist being > -2 units throughout the follow-up period. Clinical and radiographic data obtained during follow-up were recorded for all patients. RESULTS: Of a total of 250 patients for whom we had approximately 900 wrist radiographs, 6 patients were found to have unilateral erosive wrist synovitis. The JIA onset subtype was oligoarticular in 5 patients and polyarticular in 1 patient and the disease duration from presentation to the last follow-up visit ranged from 2 to 16 years. The arthritis course was polyarticular in all patients. Five patients had positive antinuclear antibodies (ANA) and 1 had positive rheumatoid factor (RF). At the last follow-up visit, all patients had some impairment of wrist function and 2 patients had wrist subluxation. There was a marked radiographic damage in all affected wrist, with the Poznanski ranging from -8.0 to -8.50 units in 3 patients and being -5.5, -3.1 and -2.4 units, respectively, in 3 patients. The severity of radiographic damage in the ANA-positive patients with the longest disease duration was comparable to that observed in the RF-positive patient. CONCLUSION: Unilateral erosive wrist synovitis seems to be uncommon in JIA. Patients with unilateral wrist synovitis may be at risk of a destructive course irrespective of the JIA onset subtype.

Arthritis, Juvenile↗
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Inter-observer agreement of the physician's global assessment of disease activity in children with juvenile idiopathic arthritis.

OBJECTIVE: To assess the inter-observer agreement in the physician's global assessment of overall disease activity (MD global) in a cohort of patients with juvenile idiopathic arthritis (JIA). METHODS: Forty consecutive patients with JIA, who were representative of a wide spectrum of disease activity and severity, were examined simultaneously by 4 observers. Observer 1 (who was the most experienced rheumatologist) carried out a routine rheumatologic examination of each patient including a complete articular assessment, and subsequently calculated in secrecy the MD global score on an anchored horizontal 10-cm VAS. Observers 2, 3 and 4 were present during the examination; afterwards they also scored in secrecy the MD global score for the patient. Agreement was measured by the intra-class correlation coefficient (ICC), using the score of Observer 1 as the gold standard. An ICC below 0.75 was considered unsatisfactory. RESULTS: The mean (SD) MD global scores for Observers 1, 2, 3, and 4 were 5.2 (3.4), 6.7 (3.9), 5.9 (3.5), and 5.6 (3.7), respectively. The level of agreement with Observer 1 in scoring was 0.83 for Observer 2, 0.88 for Observer 3, and 0.90 for Observer 4, indicating good agreement for all observers. CONCLUSIONS: Our study shows a good inter-observer agreement in the physician's global assessment of overall disease activity in patients with JIA. Analyses involving investigators from different countries are needed to determine whether these results can be generalized.

Adolescent↗
PubMedDate not supplied

Responsiveness of clinical measures to flare of disease activity in juvenile idiopathic arthritis.

OBJECTIVE: To compare the responsiveness of clinical measures in the assessment of disease flare in patients with juvenile idiopathic arthritis (JIA). METHODS: The clinical records of all consecutive patients with JIA who were diagnosed between 1995 and 2000 were retrospectively reviewed. In each patient, all visits made during follow-up were analyzed and those meeting the criteria for disease flare were recorded. The definition of flare was based on the therapeutic alterations made by the attending physician. Responsiveness of JIA clinical measures to relevant increase in disease activity (a flare) was evaluated by assessing the score change of each measure from a visit made 6 (+/- 3) months before a flare and the flare visit. Responsiveness statistics included the standardized response mean (SRM) and the effect size (ES). RESULTS: A total of 115 patients, who were followed for 0.5 to 6.2 years (mean 2.8 years), were studied. During follow-up, 51 patients (44%) experienced 1 or more disease flares, with the total number of flares being 75. Strong responsiveness (ES and SRM > or = 0.8) to increase in disease activity was demonstrated by the physician's and parent's global assessments, the global articular severity score, and the morning stiffness. The active, swollen and painful joint counts, the swelling, pain on motion/tenderness and limited range of motion (LROM) scores, and the erythrocyte sedimentation rate revealed moderate responsiveness (ES and SRM > or = 0.5). The poorest performances (ES and/or SRM < 0.5) were provided by the parent's assessment of pain, the functional ability tool, the number of joints with LROM, the LROM score, the C-reactive protein, the white blood cell and platelet count, and the hemoglobin level. CONCLUSION: Our analysis suggests that the swollen or painful joint counts are better suited than the count of joints with LROM for the assessment of disease flare in patients with JIA.

Arthritis, Juvenile↗
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Remission in juvenile idiopathic arthritis.

Until recently, no uniform and widely accepted criteria for defining remission in juvenile idiopathic arthritis (JIA) were available. In recent years, a set of preliminary criteria for clinical remission in JIA was developed through an international collaborative effort. These criteria enable the classification of patients in the states of inactive disease, clinical remission with medication, and clinical remission without medication. The first phase of the validation process of the criteria, which was accomplished recently, established that they are feasible and have good face, content and construct validity, and strong discriminant properties. A few studies have applied the new remission criteria in series of patients with JIA, with results that concur with those of previous surveys in showing that only a few patients with JIA have a chance of remaining in long-term remission status without medications. These findings highlight the critical need for therapies that have the capacity to induce sustained complete disease control of JIA.

Arthritis, Juvenile↗
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Cyclosporine A in juvenile idiopathic arthritis. Results of the PRCSG/PRINTO phase IV post marketing surveillance study.

OBJECTIVE: To investigate the clinical use patterns, clinical effect and safety of cyclosporine A (CSA) in juvenile idiopathic arthritis (JIA) in the setting of routine clinical care. METHODS: An open-ended, phase IV post marketing surveillance study was conducted among members of the Pediatric Rheumatology Collaborative Study Group (PRCSG) and of the Paediatric Rheumatology International Trials Organisation (PRINTO) to identify patients with polyarticular course JIA who had received CSA during the course of their disease. RESULTS: A total of 329 patients, half of whom had systemic JIA, were collected in 21 countries. Data were collected during 1240 routine clinic visits. CSA was started at a mean of 5.8 years after disease onset and was given at a mean dose of 3.4 mg/kg/day. The drug was administered in combination with MTX in 61% and along with prednisone in 65% of the patients who were still receiving CSA. Among patients who were still receiving CSA therapy at the last reported visit, remission was documented in 9% of the patients, whereas in 61% of the patients the disease activity was rated as moderate or severe. The most frequent reason for discontinuation of CSA was insufficient therapeutic effect (61% of the patients); only 10% of the patients stopped CSA because of remission. In 17% of the patients, side effects of therapy was given as the primary reason for discontinuation. CONCLUSION: This survey suggests that CSA may have a less favourable efficacy profile than MTX and etanercept, whereas the frequency of side effects may be similar. The exact place of CSA in the treatment of JIA can only be established via controlled clinical trial.

Antirheumatic Agents↗
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Elevated IgA anti-gliadin antibodies in juvenile chronic arthritis.

Increased intestinal permeability secondary to treatment with non-steroidal anti-inflammatory drugs (NSAIDs) and raised levels of anti-gliadin antibodies (AGA) have been reported in adults with rheumatoid arthritis. We have therefore retrospectively investigated the presence of serum AGA of the IgA and IgG classes in 70 patients with juvenile chronic arthritis (JCA). Serum IgA (but not IgG) AGA were found to be higher in JCA patients than in controls (6.2 +/- 8.7 vs 2.1 +/- 1.5 AU/ml; p less than 0.0001). This finding was observed independently of the JCA onset subtype or disease activity; however, lower levels of IgA AGA were found in patients with pauciarticular JCA and in those in remission. No significant differences in IgA AGA serum levels were observed between untreated patients and patients treated with NSAIDs. Five patients who presented the highest levels of IgA AGA were further studied a second time; serum IgA AGA were found to be markedly reduced or normalized and no clinical or laboratory evidence of coexistent coeliac disease was observed. In conclusion, our results suggest that the elevation of IgA AGA seen in our patients is secondary to non-specific immune stimulation rather than to an NSAID-induced increase in intestinal permeability.

Adolescent↗
PubMedDate not supplied

[Juvenile dermatomyositis].

Juvenile dermatomyositis (JDM) is a chronic multisystem inflammatory disease that primarily involves skin and muscles. The clinical picture is mainly characterized by the typical cutaneous rash and by skeletal muscle weakness. Nevertheless the vasculitic process may extend in some cases to other organs such as lung, joints or gastrointestinal system. Calcinosis represents a relatively frequent complication of JDM and occurs in most cases during the late stage of the disease. Usually serum levels of muscle enzymes are elevated and electromyography shows signs of myositis. Muscle biopsy is generally performed only in doubtful cases. JDM should be differentiated from the muscle involvement of other diffuse inflammatory connective tissue diseases and from non inflammatory myopathies. The treatment is mainly based on steroids, whose dosage and regimen depend on disease severity. Steroid therapy has greatly improved the prognosis of JDM.

Adolescent↗
PubMedDate not supplied

Anticardiolipin syndrome in childhood: a report of two cases.

We describe two children with an otherwise unexplained deep vein thrombosis associated with high titer anticardiolipin antibodies (ACA) of the IgG class and circulating lupus anticoagulant (LAC). One of these patients had persistent proteinuria but neither had systemic lupus erythematosus. Our observation suggests that ACA and LAC assays should be performed in children with thromboembolic disease even when no underlying autoimmune disease can be found.

Adolescent↗
PubMedDate not supplied

Multiple inhibitors of mitogen-induced proliferation of normal lymphocytes in juvenile chronic arthritis sera.

We evaluated the effect of 47 serum samples obtained from 34 children with juvenile chronic arthritis (JCA) on mitogen-induced proliferation of normal peripheral blood lymphocytes (nPBL). We found that sera from patients with active disease, and particularly those with the systemic form, inhibited significantly PHA-induced proliferation of nPBL at all the PHA concentrations tested. This inhibitory activity was independent of the treatment and was correlated with the value of the erythrocyte sedimentation rate. Part of the JCA sera inhibitory effect could be reversed by the addition of exogenous interleukin 2 (IL-2), but not of interleukin 1 (IL-1) or interferon-gamma, moreover, JCA sera were able to partially inhibit the IL-2 dependent proliferation of CTLL. When we tested serum fractions obtained by Sephadex G-200 chromatography for their inhibitory activity, we observed that: a) two major peaks of inhibitory activity on PHA-induced proliferation were present: peak 1 with MW less than 600,000 and peak 2 with MW between 70,000 and 35,000; b) the inhibitory activity present in the high MW peak was in part IL-2 related; and c) both peaks contained elevated levels of acute phase proteins (APP) which are known to inhibit mitogen-induced lymphocyte proliferation. We conclude that sera from patients with active systemic JCA contain inhibitory activity on mitogen-induced lymphocyte proliferation. We conclude that sera from patients with active systemic JCA contain inhibitory activity on mitogen-induced lymphocyte proliferation. This activity is due to the presence of multiple inhibitors, one of which appears to be IL-2 related; at least part of the inhibitory activity may be due to elevated serum levels of APP.

Adolescent↗
PubMedDate not supplied

[Changes in the production of IgM in response to pokeweed mitogen in juvenile rheumatoid arthritis. Correlations with disease activity].

Unstimulated and pokeweed mitogen (PWM)-stimulated "in vitro" IgM synthesis by peripheral blood lymphocytes has been evaluated by an ELISA method in 33 patients with juvenile rheumatoid arthritis (JRA). Lymphocyte PWM responses were significantly reduced in patients with JRA compared with healthy controls. When patients were divided according to disease activity, a significant increase of stimulation index (ratio of IgM synthesis with over that without PWM) has been observed in patients with disease remission compared with those with active disease. These findings confirm the defect in PWM-induced IgM synthesis by peripheral blood lymphocytes of patients with JRA and suggest that these abnormalities may be related to disease activity.

Adolescent↗
PubMedDate not supplied

Autoantibodies in juvenile dermatomyositis.

Fourteen patients with juvenile dermatomyositis (JDM) have been investigated for the presence of several serum autoantibodies: antinuclear (ANA), anti-single-stranded and double-stranded DNA, anti-histones, anti-Sm, anti-ribonucleoprotein, anti-SSA/SSB, anti-PM-1, anti-Jo-1, anti-mitochondrial, anti-smooth muscle, anti-gastric parietal cells, anti-cardiolipin (ACA) antibodies and rheumatoid factor. Patients were negative for all autoantibodies except for ANA and ACA. ANA were detected in 50% of the patients when tested on rat liver, but the percentage of positivity rose to 86% when HEp-2 cells were used as substrate. This finding suggests that HEp-2 cells represent a more sensitive substrate than rat liver for the detection of ANA in JDM. Three patients were positive for ACA; two of these presented vascular complications, thus suggesting a possible relationship between ACA and vascular involvement in JDM.

Antibodies, Antinuclear↗