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Infantile systemic juvenile rheumatoid arthritis (JRA) is challenging to diagnose. A 7-month-old patient with JRA showed significant symptom control with alternate-day ACTH-Z therapy when other treatments failed.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Systemic juvenile rheumatoid arthritis (JRA) presents with chronic arthritis and extra-articular manifestations.
- Onset during the first year of life (infantile JRA) is uncommon and diagnosis is difficult due to limited patient complaints.
Observation:
- A 7-month-old female experienced daily fevers and rash starting at 6 months, progressing to systemic JRA.
- Standard treatments including aspirin, NSAIDs, and prednisone were ineffective in managing her severe symptoms.
Findings:
- Alternate-day administration of ACTH-Z (4 units) was initiated.
- This regimen demonstrated significant clinical improvement and symptom control in the patient.
Implications:
- ACTH-Z may be a viable therapeutic option for managing difficult cases of infantile JRA.
- This suggests a potential need for adjusted treatment strategies in pediatric versus adult JRA patients.
Abstract:
Systemic juvenile rheumatoid arthritis is characterized by chronic arthritis and associated with extra-articular features. Although JRA may present at any age throughout childhood, the onset of symptoms during the first year of life is relatively uncommon. The diagnosis of infantile JRA is difficult because of poor complaints. We had a 7-month-old female, first onset at 6 months of age for daily "spiking" fever and skin rash, subsequently developed signs of systemic rheumatoid arthritis. Although aspirin or other nonsteroidal anti-inflammatory agents were used for the therapy, no significant effect was obtained. Prednisone was administered to control arthritis and serious systemic features, however no satisfactory effect was shown. In this patient a single morning alternate-day shot of ACTH-Z (4 units) was employed, and this regimen showed significant effect to control her clinical symptoms. It may be necessary to add a small dose of ACTH-Z for control in some cases of infantile JRA patient compared to adult cases.