Abstract

In order to establish if neuropsychiatric systemic lupus erythematosus (NPSLE) can be identified by any characteristic other than those used to diagnose the neuropsychiatric (NP) disease itself, we retrospectively reviewed 98 systemic lupus erythematosus (SLE) patients followed over a mean period of 10 years. NPSLE was identified in 22 patients. Stroke and generalized seizures were the most frequent NP manifestations. The NPSLE and non-NPSLE groups were similar with regard to demographic characteristics, ACR criteria, serum autoantibodies, and frequency of hypertension and hypercholesterolemia. Of note, compared to the non-NPSLE group, NPSLE was associated with a higher frequency of smoking (78 versus 26%), organ damage (73 versus 34%), and cumulative mortality rate (14 versus 7%). The series of patients was further analysed according to the presence of antiphospholipid syndrome (APS). Significantly, the interval between the onset of NP disease and SLE diagnosis was shorter in the APS− (0.3 ± 1 years) than in the APS+ (5 ± 7 years) groups. Recurrence and/or persistence of NP events were only documented in the APS− group. Overall cumulative mortality was highest in NPSLE and in APS+ patients with inadequate anticoagulation control, identifying an aspect that requires improved vigilance and the development of novel therapeutic modalities.

Highlights

  • In the course of their disease, many patients with systemic lupus erythematosus (SLE) develop neurologic and psychiatric symptoms

  • Anti-cardiolipin, antiβ2-GPI, or lupus anticoagulant (LAC) was present in 43% of all SLE patients, and the frequency was similar in neuropsychiatric SLE (NPSLE) and non-NPSLE

  • The objective of this work was to find features beyond those associated with the clinical diagnosis of NPSLE that may characterize this subgroup of patients

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Summary

Introduction

In the course of their disease, many patients with systemic lupus erythematosus (SLE) develop neurologic and psychiatric symptoms. The proportion of NP cases amongst SLE patients may be overestimated because events such as cognitive impairment, mood, anxiety disorders, and headaches depend on assessing the subjective complaints of patients and are very frequent in the general population. The American College of Rheumatology (ACR) has listed 19 clinical entities that define NPSLE [4], but these do not differentiate, in population-based studies, NPSLE patients from non-SLE controls. NP events attributed to SLE occur mainly in the 6 months prior to, and in the first year following the diagnosis of SLE. These may be observed as late as 15 years after the initial diagnosis of SLE [6]. The life expectancy of patients with SLE has significantly improved over the past 50 years [7,8,9,10], NPSLE patients have a poorer quality of life than non-NPSLE patients [3, 6]

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