儿童期发病的系统性红斑狼疮的初期表现:法国的多中心研究  

Initial presentation of childhood-onset systemic lupus erythematosus: A French multicenter study

作　　者：Bader-Meunier B. Armengaud J.B. Haddad E. 李丹 

机构地区：[1]Serv . de Pêdiat. Gên., Hpital de Bicêtre, 78 rue du Gên. Leclerc, 94276 Le Kre mlin Bicêtre, France Dr.

出　　处：《世界核心医学期刊文摘（儿科学分册）》2005年第10期56-56,共1页

摘　　要：Objective: To describe the clinical and laboratory 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 before 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 abdominal involvement in 26 patients, which le ad to negative laparotomy results for presumed appendicitis. Severe renal, neuro logic, hematologic, abdominal, cardiac, pulmonary, thrombotic, 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 antibodies an, anti-double s tranded DNA antibodies, 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 be promptl y considered in any febrile adolescent with unexplained organ involvement, espec ially when associated with an increased erythrocyte sedimentation rate.Objective: To describe the clinical and laboratory 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 before 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 abdominal involvement in 26 patients, which le ad to negative laparotomy results for presumed appendicitis. Severe renal, neuro logic, hematologic, abdominal, cardiac, pulmonary, thrombotic, 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 antibodies an, anti-double s tranded DNA antibodies, 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 be promptl y considered in any febrile adolescent with unexplained organ involvement, espec ially when associated with an increased erythrocyte sedimentation rate.

关 键 词：系统性红斑狼疮 童期 肺部表现 红细胞沉降 抗核抗体 血栓形成 发病年龄 腹部症状 研究设计 DNA 

分 类 号：R725.9[医药卫生—儿科]