[Systemic juvenile idiopathic arthriris: diagnosis and management]

ABSTRACT

Systemic juvenile idiopathic arthritis is a relatively rare disease in childhood. Sometimes, difficulties are encountered because it is a diagnosis of exclusion. The authors describe the clinical, biologic, therapeutic and evolutive characteristics of this affection. It is a retrospective study during a period of 16 years from 1990 to 2005. Inclusion criteria respond to Durban criteria, after exclusion of other etiologies of febrile eruptions. The authors analyse epidemiologic and clinical characteristics of the disease, biologic exams, treatment and evolution. Nine eases are reported during study period. Patients are between 22 month and II year old. Biologic analysis shows an important inflammatory syndrome with a sedimentary rate superior to 80 mm at first hour in average, a white blood cell count superior to ll.000/mm3. the evolution under treatment is characterized by resistance to steroids in one case and dependence to steroids in 2 eases. The complications include a macrophage activation syndrome in 2 cases and complications secondary to steroids in one ease. Systemic juvenile idiopathic arthritis remains a relatively rare disease in childhood. It's management is improved by the emergency of new treatments bnt its evolution is unpredictable