Objective: To determine the prevalence and characteristics of central nervous system disease in systemic lupus erythematosus (CNS/SLE) with particular reference to disease activity and to the presence of antiphospholipid (aPL) antibodies.
Patients and methods: From 340 unselected SLE patients attending our lupus clinic, we found 96 (28%) to have definite CNS manifestations not attributed to any cause other than SLE. Patients with mild migraine or cognitive disorders were excluded. The control group consisted of 100 SLE patients without CNS or thromboembolic manifestations.
Results: Fifty-six of our CNS patients had transient ischemic attacks or strokes, 24 had epilepsy, and 12 had psychiatric disorders; the other 4 did not fulfill SLE criteria. In all, 55% of patients (53) were found to be positive for aPL antibodies, whereas only 20% of the SLE control group were positive (P < 0.001). Based on a physicians' global clinical assessment tool together with laboratory analysis, only 42 (44%) patients were found to be active at the onset of CNS manifestations, and the other 54 (56%) were nonactive. A finding of aPL antibodies was associated strongly with the inactive CNS/SLE group (P = 0.001). Of the 53 patients who underwent magnetic resonance imaging (MRI) study, 33 showed small high-density lesions suggestive of vasculopathy. Twenty-six (79%) of them were positive for aPL antibodies; whereas of the 20 patients with normal MRIs, only 8 (40%) were positive for aPL antibodies (P < 0.01).
Conclusion: We confirm that CNS disease in SLE is significantly associated with the presence of aPL antibodies. The CNS manifestations can occur in about half of SLE patients without any other evidence of lupus activity. Abnormal MRIs highly correlate with positive aPL antibodies.