Challenges in the diagnosis and treatment of systemic juvenile idiopathic arthritis: A case report
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Abstract
Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by early predominant systemic manifestations (e.g., prolonged fever, evanescent rash, hepatosplenomegaly, and lymphadenopathy), followed by chronic arthritis. Confirm diagnosis is often delayed because of challenges in distinguishing sJIA from other causes of prolonged fever in children, the lack of specific diagnostic biomarkers, and the limitations of current diagnostic criteria. We report the case of a 7-year-old male presenting with prolonged high-grade fever, fever-associated rash, arthritis, and significantly elevated inflammatory markers. The patient was initially misdiagnosed at other hospitals with acute infectious urticaria (unresponsive to antibiotic therapy) and subsequently with oligoarticular JIA (refractory to a 4-month course of glucocorticoids and methotrexate). We established a diagnosis of sJIA and achieved clinically inactive disease with tocilizumab. We also discuss classification criteria that facilitate early diagnosis and recent changes in treatment approaches for sJIA.
Article Details
Keywords
Systemic juvenile idiopathic arthritis, Still’s disease, diagnosis, treatment
References
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