Clinical analysis of neuropsychiatric systemic lupus erythematosus involving the central nervous system

Abstract

Background Neuropsychiatric systemic lupus erythematosus (NP-SLE) presents with a wide variety of clinical manifestations, which is often difficult to diagnose with a high mortality. This study aims to investigate the clinical features of NP-SLE involving the central nervous system (CNS) and the differential diagnoses between CNS NP-SLE and intracranial infections. Methods The clinical manifestations, serum immunological features, cerebrospinal fluid (CSF) examinations (including intracranial pressure, leukocyte count, protein, glucose and chloride), CT and (or) MRI and electroencephalogram (EEG) data of 23 NP-SLE patients with CNS involved were retrospectively reviewed. Results Nine patients presented with diffuse manifestations, while 14 patients presented with focal manifestations. Serum analysis showed the positive rates of immunoglobulins anti-nuclear antibody (ANA), anti-double stranded DNA antibody (dsDNA), anti-Sm, anti-ribosmal P protein, anti-SSA and anti-SSB antibodies were 21/22, 7/22, 1/14, 2/14, 9/14 and 3/14 respectively. Patients with decreased serum C3 accounted for 14/20 while patients with decreased serum C4 accounted for 5/20. Besides, patients with increased CSF leukocyte count and microalbumin took up 5/12 and 7/12, while patients with decreased glucose and chloride levels took up 5/12 and 6/12. All 23 patients presented abnormal CT and (or) MRI and 6 patients presented abnormal EEG. Conclusion Serum immunological levels, CT and (or) MRI and EEG examinations contributed to the diagnosis of NP-SLE involving CNS. Although CSF analyses were slightly abnormal, the increase of leukocyte count and average microalbumin was not obvious, and the mean values of glucose and chloride were in the normal range, suggesting that the CSF examinations were helpful for the differential diagnoses from intracranial infections. Glucocorticoids and immunosuppressive drugs were remarkably effective for CNS NP-SLE patients.