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[Juvenile idiopathic systemic arthritis]
Dalia Baksiene1, Jūrate Kasparaviciene, Migla Zebiene
1Clinics of Pediatrics, Kaunas University of Medicine, 3007 Kaunas, Lithuania. childdgn@kmu.lt
Insights
Systemic juvenile idiopathic arthritis presents with prolonged fever and rash. Intravenous methylprednisolone pulses are a safe and effective treatment for most children with this condition.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Systemic juvenile idiopathic arthritis (sJIA) is a rare autoimmune disease.
- sJIA diagnosis relies on clinical and laboratory findings, with no specific biomarkers.
- Early and accurate diagnosis is crucial for effective management.
Purpose of the Study:
- To evaluate clinical features, laboratory parameters, and treatment strategies for sJIA.
- To analyze the presentation and progression of sJIA in pediatric patients.
- To assess the efficacy and safety of methylprednisolone pulse therapy in sJIA.
Main Methods:
- Retrospective data review of 41 children diagnosed with sJIA between 1992 and 2002.
- Analysis of clinical symptoms, laboratory results, and treatment regimens.
- Evaluation of outcomes following intravenous methylprednisolone pulse therapy.
Main Results:
- All patients presented with prolonged fever of unknown origin; 49% had a maculo-papular rash.
- Common findings included neutrophilic leukocytosis (73%), anemia (80.5%), and elevated CRP (63.4%).
- Intravenous methylprednisolone pulse therapy was effective in most cases, with 24.4% requiring additional immunosuppressants.
Conclusions:
- Systemic juvenile idiopathic arthritis often presents with persistent fever and characteristic rash.
- Methylprednisolone pulse therapy is a safe and effective initial treatment for sJIA.
- A subset of patients may require combination therapy with immunosuppressive agents.
Unlabelled:
THE PURPOSE OF THE STUDY was to evaluate the peculiarities of the clinical features, laboratory parameters and tactics of treatment in juvenile idiopatic systematic arthritis.
Methods:
A retrospective data review of 41 children (26 boys and 15 girls) who underwent treatment for systemic arthritis (according to ILAR criteria) in our institution between 1992 and 2002 was performed.
Results:
The disease started with fever of unknown origin in all cases. In 73% of patients it lasted longer than one month, in 54% fever was with twice daily spikers in the morning and in the evening. The rash during the rise of temperature appeared in 49%, in most cases (70%) there was a maculo-papular rash. Lymphadenopathy and serositis were observed in 32%, hepatomegaly in 29%, and splenomegaly in 24%. Arthritis coincided with the fever in 29% of patients, in majority of cases it was progressing to a severe persistent arthritis after the systematic phase. There was no specific laboratory findings: neutrophilic leucocytosis was found in 73%, anemia - in 80.5%, trombocytosis - in 36.6%, elevated CRP - in 63.4%, dysproteinemia - in 79% of patients. Antinuclear factors were absent in all examined children. For all patients intravenous methylprednisolone pulses have been administered (10-22 mg/kg/infusion). Prednisolone was also continued orally (1-2 mg/kg/day). 24.4% of patients required in addition immunosupressive agents such as methotrexate, azathioprine and cyclophosphamide.
Conclusion:
Puls-therapy of methylprednisolone is a safe and sufficiently effective method of treatment in most cases of the systematic juvenile arthritis.
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