Objective To review the diagnosis and treatment of children with systemic lupus erythematosus. Methods 13 children of systemic lupus erythematosus with acute immune thrombocytopenic purpura as the first symptom were analyzed retrospectively, who were admitted from Jan 2004 to Jun 2009 in our hospital. Results Thirteen cases were diagnosed as acute immune thrombocytopenie purpura according to clinical manifestations and bone marrow examination results. Hormones and intravenous gamma globulin treatment were ineffective. The immune parameters and other clinical manifestations were monitored. According to the characteristics of blood system change and the response to hormone, other immunosuppressive agents such as cyclosporine A ,mycophenolate mofetil and cyclophosphamide were adminitrated. Thirteen children were diagnosed finally as systemic lupus erythematosus at 2 to 24 months from onset. Other immunosuppressive treatment was effective. Conclusion For children with thrombocytopenia, especially adolescent girls, we should pay attention to dynamic monitoring of anti-nuclear antibodies and other immunological parameters,for fear of the possibility of systemic lupus erythematosus.