Neuropsychiatric systemic lupus erythematosus: Lessons learned from magnetic resonance imaging

Abstract

Objective. The clinical manifestations of nervous system involvement in systemic lupus erythematosus (neuropsychiatric SLE [NPSLE]) are highly diverse, and their etiology is incompletely understood. The aim of this study was to provide an inventory of abnormalities on conventional brain magnetic resonance imaging (MRI) in NPSLE and to interpret the findings in relation to possible underlying pathogenetic mechanisms.Methods. MR images of the first episode of active NPSLE in 74 patients were retrospectively reviewed. All patients fulfilled the American College of Rheumatology (ACR) 1982 revised criteria for the classification of SLE and were classified according to the 1999 ACR case definitions for NPSLE syndromes. We excluded patients with a history of brain disease and patients in whom other mechanisms unrelated to SLE caused the neuropsychiatric symptoms.Results. The principal findings were: 1) focal hyperintensities in white matter (WM) (49% of all patients) or both WM and gray matter (GM) (5% of all patients), suggestive of vasculopathy or vasculitis; 2) more widespread, confluent hyperintensities in the WM, suggestive of chronic hypoperfusion due to the same mechanisms; 3) diffuse cortical GM lesions (12% of all patients), compatible with an immune response to neuronal components or postseizure changes; and 4) absence of MRI abnormalities, despite signs and symptoms of active disease (42% of all patients).Conclusion. Several distinct brain MRI patterns were observed in patients with active NPSLE, suggestive of different pathogenetic mechanisms. To advance our understanding of the various processes leading to NPSLE, the radiographic manifestations may be a good starting point and useful for categorization of patients in further research.In the course of their disease, many patients with systemic lupus erythematosus (SLE) develop neurologic or psychiatric symptoms. After exclusion of other causes such as concomitant illnesses, infection, or drug side effects, these neuropsychiatric manifestations are attributed to involvement of the nervous system in SLE, which is referred to as neuropsychiatric SLE (NPSLE) (1). Correct attribution of neuropsychiatric events to NPSLE or to an alternative etiology is a challenge, given the absence of a diagnostic gold standard for NPSLE. In clinical practice, NPSLE is a diagnosis per exclusionem, achieved case-by-case using clinical, laboratory, and imaging data (1,2). Consequently, the diagnosis is inevitably presumptive. Magnetic resonance imaging (MRI) is the imaging technique of choice in the diagnosis of NPSLE (2,3). It is widely available and permits identification of lesions associated with NPSLE and many differential disorders.NPSLE comprises a wide range of clinical conditions affecting the central, peripheral, or autonomic nervous system, such as cognitive dysfunction, psychosis, depression, and acute confusional state, as well as more focal syndromes, such as stroke, seizures, chorea, or transverse myelitis (4). The severity of the symptoms is also highly variable. T...

Comparison of structural magnetic resonance imaging findings between neuropsychiatric systemic lupus erythematosus and systemic lupus erythematosus patients: A systematic review and meta-analysis

“…An underlying acute infarct has been suggested in areas where reduced diffusion was observed secondary to cytotoxic oedema, while bilateral WMHs are thought to be a sign of chronic hypoperfusion. 20 Common sites of WMH lesions are in the frontoparietal region, periventricular and subcortical white matter, and their presence has been recorded in both active and inactive NPSLE. 29,[47][48][49] Although evidence still persists that WMHs are not specific to NPSLE, as it was shown in SLE without overt neuropsychiatric involvement, they were found to be higher in quantity and total volume in active and past NPSLE when compared with non-NPSLE patient groups.…”

“…Third, there is a chance that some of the NPSLE syndromes may (e.g. anxiety) develop later 19 Luyendijk et al 20 and Jennings et al 21 Fluid-attenuated inversion recovery (FLAIR)…”

“…Subcortical and periventricular WMH lesions; infarcts Luyendijk et al, 20 Yaniv et al 22 and Ercana et al 23 …”

“…46 A variety of MRI investigation descriptions were provided. In 10 papers, T1, T2 and fluid-attenuated inversion recovery (FLAIR) were used; 20 25 and MRI, SPECT. 42 Five studies were undertaken in the United States, 21,36,41,44,45 three in Japan, 34,35,46 two in South Korea, 25,32 two from the Netherlands 20,24 and two from Brazil.…”

Magnetic Resonance Imaging in Neuropsychiatric Lupus

“…In a recent study in patients with newly diagnosed SLE, WMH were found in 8% of the patients 38 . Nevertheless, these lesions were observed more frequently in NPSLE when compared with SLE without neuropsychiatric manifestations, with average ranges from 40 to 60% 8, 11, 19, 34, 36, 38, 39 . WMH were associated with cerebrovascular disease, cognitive dysfunction, seizures, antiphospholipid antibodies, low complements (C3, C4, CH50), age, disease duration, and total corticosteroid dose 8, 34 .…”