title: Juvenile idiopathic arthritis presenting with prolonged fever.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
authors.forename: Tzu-Lin
authors.initials: TL
authors.lastname: Yeh
authors.forename: Fu-Yuan
authors.initials: FY
authors.lastname: Huang
authors.forename: Shyh-Dar
authors.initials: SD
authors.lastname: Shyur
authors.forename: Tien-Ling
authors.initials: TL
authors.lastname: Chen
authors.forename: Chyou-Shen
authors.initials: CS
authors.lastname: Lee
authors.forename: Daniel Tsung-Ning
authors.initials: DT
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001171:Arthritis, Juvenile / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D001424:Bacterial Infections / Q000175:diagnosis; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005076:Exanthema; D005260:Female; D005334:Fever / Q000209:etiology*; D006529:Hepatomegaly; D006801:Humans; D007223:Infant; D008206:Lymphatic Diseases; D008297:Male; D013163:Splenomegaly; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D018501:Antirheumatic Agents; D013256:Steroids; D002097:C-Reactive Protein