Biomedical subjects

B Ramenghi

Publications and source records attributed to B Ramenghi.

10 recordsLinked to original sources

PubMed1998

Radiologic progression in patients with juvenile chronic arthritis treated with methotrexate.

We investigated the rate of radiologic progression, as measured by carpal length, in 26 children with juvenile chronic arthritis who were treated with methotrexate. After 2 years of treatment, the carpal length was significantly more deteriorated in nonresponders in both the "better" wrist (P = .001) and the "worse" wrist (P = .005). These results show that methotrexate may have a "disease modifying" potential in juvenile chronic arthritis.

Adolescent↗

PubMed1996

Pulmonary function in children with systemic lupus erythematosus.

BACKGROUND: Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. METHODS: After a mean of 4.5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). RESULTS: In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage of patients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. CONCLUSIONS: In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease.

Adolescent↗

PubMed1995

Evaluation of response to methotrexate by a functional index in juvenile chronic arthritis.

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 (p < 0.001); 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.

Adolescent↗

PubMed1995

Frequency of relapse after discontinuation of methotrexate therapy for clinical remission in juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the outcome of children with juvenile rheumatoid arthritis (JRA) who discontinued taking methotrexate (MTX) therapy after the achievement of clinical remission. METHODS: We conducted a retrospective review of the clinical course of all consecutive patients with JRA treated with MTX at our department. RESULTS: Seventeen of the 30 patients who responded to treatment entered clinical remission 6 to 30 mo after beginning MTX therapy. All patients were discontinued from MTX within 2 to 5 mo. Five patients relapsed within 9 mo, whereas 12 patients had sustained remission for 12 mo or longer. Four of 5 children with extended pauciarticular JRA relapsed early after MTX discontinuation. These patients were less responsive to a further cycle of MTX, and 3 showed progression of radiographic joint lesions. CONCLUSION: These findings suggest that MTX should be continued in patients with extended pauciarticular JRA even after the achievement of clinical remission.

Adolescent↗

PubMed1994

Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis.

We retrospectively investigated, in 19 children with systemic-onset juvenile chronic arthritis (SoJCA), the possible influence on the outcome of methotrexate (MTX) therapy of several independent variables, including age at onset of juvenile chronic arthritis, disease duration and severity of the disease at baseline. The dosage of MTX ranged from 7.5 to 11.0 mg/m2/week (median 9.3 mg/m2/week) and was given as a single, oral weekly dose. After 6 months of treatment, 12 (63%) patients were judged as responders on grounds of a > or = 50% reduction in the number of joints with active arthritis and/or an articular severity score; 7 (37%) did not respond to therapy. When the baseline values of the selected variables were compared, we found that the responder group had, with respect to the non-responder group, a lower percentage of radiographic lesions (p < 0.005), a shorter duration of the disease (p < 0.05) and a lower number of joints with limitation of motion (p < 0.01), functional limitation score (p < 0.05) and articular severity score (p < 0.05). A threshold value of disease duration of two years and the presence/absence of radiographic lesions gave a correct classification with respect to the treatment outcome of 73.7% and 83.3%, respectively. The predictive value of these two variables was confirmed by a multivariate analysis. We conclude that earlier treatment with MTX, possibly before the appearance of radiographic changes, may favourably influence the outcome of MTX treatment in those patients with SoJCA who require a second-line drug.

Adolescent↗

PubMed1993

Plasma levels after oral methotrexate in children with juvenile rheumatoid arthritis.

Plasma levels of methotrexate (MTX) after oral administration of 6.4 to 11.2 mg/m2/week (mean 8.5 mg/m2/week) were studied in 33 children with severe juvenile rheumatoid arthritis (JRA). MTX concentrations were measured by a fluorescence polarization immunoassay (TDx) at 1, 2, 3, and 24 h after administration. The maximum level was observed in most patients after 1 h. No significant correlation was found between MTX dosage and the 1, 2, and 3-h plasma levels. No patient showed values in the range of probable toxicity 24 h after administration. A stepwise multiple regression analysis on 1, 2, and 3-h MTX levels, selected clinical features, dosage and duration of MTX therapy, and concomitant drug treatment showed that MTX concentrations at the different time points tend to be closely related; among the other variables, only concurrent treatment with salicylates was found to affect significantly the 3-h level. Serial determinations performed in 20 patients at the same oral dosage showed a wide interindividual and intraindividual variability of the plasma levels from the first dose to the next. Variable and unpredictable levels were observed also in most of the 8 patients studied after one or more increases of MTX dosage. No difference in MTX concentrations was observed between patients who responded to treatment and those who failed to respond, and between patients who had serum transaminase elevation and those who did not. Our results suggest that, until the pharmacokinetics of low dose MTX is clarified, routine therapeutic monitoring of MTX has a limited value in the clinical management of children with JRA.

Administration, Oral↗

PubMed1993

Association of methotrexate treatment with a decrease of double negative (CD4-CD8-) and gamma/delta T cell levels in patients with juvenile rheumatoid arthritis.

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.

Adolescent↗

PubMed1992

Antiphospholipid antibody associated thrombosis in juvenile chronic arthritis.

A child with systemic onset juvenile chronic arthritis (JCA) who developed a bilateral femuropopliteal vein thrombosis after plaster immobilisation following a tibial fracture is described. When the thrombosis was diagnosed, antiphospholipid antibodies detected either as lupus anticoagulant and anticardiolipin antibodies were found. This suggests that short term prophylatic antithrombotic treatment should be considered in antiphospholipid antibody positive JCA patients who require immobilisation after fractures of demineralised bones.

Antibodies, Antiphospholipid↗

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

IgG autoantibodies to complement C1q in pediatric-onset systemic lupus erythematosus.

OBJECTIVE: Antibodies to C1q (aC1q) have been found in up to 50% of adult patients with systemic lupus erythematosus (SLE) and have been associated with proliferative glomerulonephritis. We investigated the prevalence and clinical significance of aC1q in pediatric SLE. METHODS: Antibodies to C1q, measured by an ELISA method, were evaluated in 29 patients with childhood-onset SLE, 26 females and 3 males, aged 7.5 to 19.6 years. RESULTS: Seventeen (59%) of the 29 patients were initially positive for aC1q. No correlation was found between either the presence or titer of aC1q and any of the clinical manifestations, including nephritis. However, a significant correlation was observed between aC1q levels and anti-double-stranded DNA antibodies and C3 values. Serial determinations of aC1q in 23 patients showed a progressive decline in the titers with few significant fluctuations. Antibodies to C1q were not a reliable predictor of increased SLE activity, since they increased or became detectable in only 50% of the pre-flare sera. CONCLUSION: Antibodies to C1q were frequently positive in our patients with pediatric SLE and were correlated with laboratory variables of disease activity. However, unlike in adults with SLE, a high correlation between aC1q and glomerulonephritis was not found in these pediatric patients.

Adolescent↗