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Volume 43, Number 3, June 2010

Juvenile Idiopathic Arthritis Presenting with Prolonged Fever


Tzu-Lin Yeh, Fu-Yuan Huang, Shyh-Dar Shyur, Tien-Ling Chen, Chyou-Shen Lee, Daniel Tsung-Ning Huang


Received: January 16, 2009    Revised: April 30, 2009    Accepted: June 22, 2009   

 

Corresponding author:

Daniel Tsung-Ning Huang

Department of Pediatrics, Mackay Memorial Hospital, 92 Section 2, Zhongshan, North Road, Taipei, Taiwan.

E-mail: zoning12huang@gmail.com




 

Background and purpose: 

 Systemic-onset juvenile idiopathic arthritis (s-JIA) is a systemic disease often accompanied by a fever. We examined 16 patients with s-JIA and reported the clinical manifestations, laboratory data, treatments and outcomes.



 

Methods:

 From 1984 to 2007, 16 children (aged 1–16 years), who were diagnosed as having s-JIA, were admitted to the Mackay Memorial Hospital in Taiwan. We retrospectively reviewed their medical charts.



 

Results:

 There were nine boys and seven girls, with mean age of onset of 7.4±5.5 years. Fever (100%), typical rash (63%), and arthritis (75%) were the three most common symptoms. Lymphadenopathy (50%), hepatosplenomegaly (63%), pleural pulmonary manifestations (13%) and myalgia (25%) were also noted. One patient had Epstein-Barr virus-associated hemophagocytic syndrome complications. Neutrophilic leukocytosis was a common feature. Other laboratory data showed elevated C-reactive protein levels (25.1±50.3 mg/dL), and erythrocyte sedimentation rates (69±28 mm/hr) and abnormal liver enzymes. Marked hyperferritinemia (> 2,000 ng/mL) was noted in 57% (4/7) of the patients. The mean time from onset of symptoms to diagnosis was 9.2 weeks. Non-steroidal anti-inflammatory drugs, steroids, disease-modifying anti-rheumatic drugs and anti-tumor necrosis factor agents were used for treatment. Due to prolonged fever, 2.0±1.6 (maximum=5) different kinds of antibiotics were used before a diagnosis was made. Most cases had satisfactory therapeutic outcomes except one boy, who had permanent joint contracture.



 

Conclusion:

 The clinical manifestations of s-JIA in Taiwan were often accompanied by a prolonged fever. This results in clinicians often suspecting bacterial infections and prescribing several kinds of antibiotics. In the case of prolonged fever, s-JIA should always be placed on the list of differential diagnoses.



 

Key words:

 clinical manifestation fever systemic-onset juvenile idiopathic arthritis