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To establish a pediatric lupus cohort with a narrow disease spectrum and assess the long-term efficacy of medication, we analyzed the clinical and immunologic characteristics of a pediatric systemic lupus erythematosus (pSLE) cohort. In addition, we were interested in the long-term effect of a relatively recent onset of disease (before age 16 years), in contrast to adult-onset disease, on the clinical and immunologic features of the disease. We consecutively assessed 183 patients (77 with pSLE and 106 with juvenile idiopathic arthritis (JIA)) for the following parameters: disease activity (Systemic Lupus Erythematosus Disease Activity Index, SLE Disease Activity Index, and Systemic Lupus Activity Measure), damage (Systemic Lupus International Collaborating Clinics Damage Index), treatment and its long-term effects, complications, and history of infections, and serum levels of inflammatory markers (erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A) and immunoglobulin isotypes (IgG, IgA, IgM, and IgD) with particular regard to autoantibody presence. The pSLE group was characterized by significantly

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