Objective: Neuropsychiatric manifestations in patients with systemic lupus erythematosus (SLE) originate from immune-mediated disease (primary neuropsychiatric SLE) or from other pathogenetic mechanisms indirectly related to SLE (secondary neuropsychiatric SLE). The objective of this study is to describe the clinical practice of diagnosing NP-SLE and to assess how often the diagnosis of primary NP-SLE is changed to secondary NP-SLE and vice versa during the follow-up period in a large series of hospitalized SLE patients.
Materials and methods: Data was collected by means of retrospective evaluation of the charts of 191 SLE patients admitted during the period 1986 to 1995.
Results: Of 191 admitted SLE patients, 49 had developed neuropsychiatric signs and symptoms. At admission 30 patients were classified as having primary NP-SLE and 19 patients secondary NP-SLE. During follow-up the diagnosis was changed to primary NP-SLE in 2 patients initially diagnosed as suffering from secondary NP-SLE, and in two patients from primary to secondary NP-SLE. Seizures, cognitive deterioration, psychosis and organic brain syndrome were the most frequent manifestations in primary NP-SLE, whereas in secondary NP-SLE headache, seizures, paresis and organic brain syndrome prevailed. 47% of the primary NP-SLE patients were re-admitted to hospital because of recurrent neuropsychiatric manifestations within 4.5 years, while 10% died due to primary NP-SLE. The prognosis of secondary NP-SLE was dependent on the diagnosis.
Conclusion: In the large majority of patients the initial diagnosis of primary or secondary NP-SLE made upon their admittance to hospital is confirmed during the long-term follow-up.