Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus

MAS can develop as the initial manifestation of jSLE. MAS with jSLE should be suspected in patients with fever, hyperferritinaemia, cytopenia and liver disorder. In addition, we found that jSLE patients with MAS had more neurological symptoms than those without. All patients with MAS were successfully treated with CSs. Early diagnosis and intensive therapy are essential in improving clinical outcomes.