摘要：Ob.ective: To describ. the clinical and lab.ratory manifestations of childhood -onset systemic lupus erythematosus (SLE) at presentation. Study design: This r etrospective French multicenter study involved 155 patients in whom SLE develope d b.fore the age of 16 years. Mean patient age at onset was 11.5 ±2.5 years (ra nge, 1.5-16 years). The female to male ratio was 4.5. Results: The most common initial manifestations were hematologic (72%), cutaneous (70%), musculoskeleta l (64%), renal (50%), and fever (58%). Thirty-two percent of children had at ypical symptoms, mainly including ab.ominal involvement in 26 patients, which le ad to negative laparotomy results for presumed appendicitis. Severe renal, neuro logic, hematologic, ab.ominal, cardiac, pulmonary, thromb.tic, and/or cutaneous manifestations occurred within the first month after the diagnosis in 40%of pat ients. The mean erythrocyte sedimentation rate was 72 ±29 mm/h, and the mean C -reactive protein value 22 ±21 mg/L. Antinuclear antib.dies an, anti-doub.e s tranded DNA antib.dies, and low C3 or C4 level were retrieved in 97%, 93%, and 78 %of patients, respectively. Conclusion: Initial manifestations of childhood -onset SLE are diverse and often severe. The diagnosis of SLE should b. promptl y considered in any feb.ile adolescent with unexplained organ involvement, espec ially when associated with an increased erythrocyte sedimentation rate.