Prognostic impact of atypical presentation in pediatric systemic lupus erythematosus: results from a multicenter study.

Objectives. The aim of the study is to assess the rate of atypical manifestations at onset in pediatric systemic lupus erythematosus (SLE) and to evaluate their effect on disease outcome. Study design. This is a multicenter retrospective cohort study. A manifestation was considered atypical if it was not included in the American College Rheumatology classification criteria but was reported in literature as associated with SLE. Conclusions. Our data report a relatively high rate of atypical onset in pediatric SLE. Presence of atypical manifestations at presentation and early kidney disease correlate with poor outcome. Similarly, during follow-up, kidney and central nervous system diseases are asociated with worse outcome.