YANG Kangkang,YE Xiaohua,SHANGGUAN Yaoyao, et al. Clinical features and prognosis of systemic-onset juvenile idiopathic arthritis[J]. JOURNAL OF WEZHOU MEDICAL UNIVERSITY, 2018, 48(10): 746-749.
Abstract：Objective: To analyze the clinical features of children with systemic-onset juvenile idiopathic arthritis (SoJIA). Methods: Patients diagnosed with SoJIA from January 2010 to January 2017 were evaluated and data were collected for clinical analysis. Results: A total of 41 patients (28 boys and 13 girls) were included in this study. The mean age of onset was (7.35± 3.17) years old. The most common presentations were fever (100%), arthritis (80.5%), and skin rash (58.5%). Macrophage activation syndrome was found in 6 cases. Oligoarticular type of arthritis accounted for 87.9% of the affected patients. The mean period from the first day of clinical symptoms to that of developing arthritis was 220 days (545 days, 112 days, 125 days, 120 days, and 200 days respectively), and the patterns of arthritis were all oligoarticular type. The most common joints involved were the knee, ankle, shoulder and elbow. Leukocytosis and anemia accounted for 68.3%, and 43.9% respectively. The majority of inflammatory parameters, including CRP, ESR and SF were elevated. 44.8% of patients had single-phase course, 17.2% had relapsing once and 37.9% had relapsing at least twice. After therapy, 25 cases achieved complete reminssion, 12 cases failed to follow-up, and 1 case was dead. Relapsing group was significantly younger. The levels of CRP, SF, ALT, CD3+, CD4+ and GGT in the relapsing group were significantly lower. Conclusion: The clinical manifestations of SoJIA were various, 12.2% of patients did not have arthritis on disease onset, but developed arthritis during follow-up process. Most prognosis is good. The younger onset age, thrombocytosis and T lymphocyte dysfunction may indicate a relapsing course.
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