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OBJECTIVE: To determine the effect of severe neuropsychiatric (SNP) manifestations on short-term damage and the time of their presentation, in young patients with systemic lupus erythematosus (SLE) of short disease duration. METHODS: One hundred thirty patients with SLE, hospitalized because of noninfectious SNP manifestations (n = 65) or other reasons (n = 65) were studied. Clinical information, including SLE characteristics, laboratory test results, treatment, disease activity, and damage, was gathered from the medical chart at 3 different dates: the index hospitalization, the closest visit prior to and one year after hospitalization. RESULTS: Demographic and SLE characteristics were comparable in patients with SNP manifestations and controls, including age at SLE diagnosis, 26.1 +/- 11.0 vs 25.7 +/- 11.2 years (p = 0.84). SNP manifestations developed early during the course of SLE, 2.5 +/- 5.2 years. At the visit prior to the hospitalization, disease activity was mild and similar in both patient groups. During hospitalization, patients who developed SNP manifestations reached higher SLE Disease Activity Index scores than controls (p < 0.0001) and also received more aggressive treatment. One year after the hospitalization, disease activity, treatment, and mortality did not differ between the 2 patient groups; however, the increase in damage was higher among the patients with SNP manifestations than controls [0.95 +/- 0.16 (95% CI 0.64-1.26) vs 0.24 +/- 0.09 (95% CI 0.07-0.41), p < 0.0001]. CONCLUSION: SNP manifestations occur early during the course of SLE and add a significant increase in damage compared to non-NP manifestations.