Optimizing clinical monitoring of central nervous system involvement in SLE
Introduction
Central Nervous System (CNS) involvement is a frequent SLE manifestation occurring in 15–75% of patients depending on population studied and inclusion criteria [1]. Normative classification criteria for NPSLE are now available representing an important tool in clinical practice [2]. However, diagnosis of CNS involvement is a difficult task and requires a careful clinical and laboratory assessment along with instrumental evaluation to rule out other causes of neuropsychiatric symptoms. About neuroimaging, it must be remembered that no single technique has diagnostic specificity for NPSLE nor accounts for all brain pathology underlying NP clinical syndromes. Furthermore, in SLE patients, clinical symptoms may occur without instrumental abnormalities and instrumental abnormalities may occur in asymptomatic patients. So far, there aren't guidelines suggesting the best neuroimaging approach to NPSLE and coupling a morphological technique with a functional technique seems actually the most valuable solution. While conventional MRI (cMRI) remains the preferred technique for the morphological evaluation of CNS in SLE patients, in the last decade major advances have been done in the assessment of perfusional, metabolic and microstructural brain alterations not detected by cMRI.
In this review we would like to summarize what have we learned in the last few years about neuroimaging in NPSLE and what have been major advances in neuroimaging techniques; finally, we also would like to give some suggestions about what should be done in daily clinical practice to approach SLE patients with NP symptoms with particular attention to the diffuse presentation of NPSLE.
Section snippets
Morphological neuroimaging
Neuroimaging techniques useful to study brain tissue morphology include magnetic resonance angiography (angio-MRI), brain angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Angiographic techniques are usually adequate for investigating medium-to-large vessels involvement and therefore are rarely applied in SLE since a small vessel vasculopathy represents the major histopathological background of brain involvement in NPSLE [3], [4], [5].
Computed tomography is a very
PET
PET is a nuclear medicine technique which explores both brain glucose metabolism and cerebral blood flow (CBF). Multiple areas of hypometabolism detected both in MRI normal-appearing white and grey matter regions (NAWM and NAGM) are the most frequent finding in NPSLE. Even if PET has high sensitivity (abnormal in 100% of patients with active NPSLE), due to it’s low specificity, limited availability, excessive cost and elevated dose radiations it’s rarely applied in daily clinical practice [9],
Perfusion weighted imaging
PWI measures regional CBF and, by using a paramagnetic contrast medium, identifies residual tissue perfusion in ischemic areas and potentially savable tissue at risk surrounding the irreversibly damaged ischemic core. Even if it is likely that this technique could be useful in NPSLE and antiphospholipid syndrome (APS) patients at risk of cerebro-vascular ischemic events, reports on the use of PWI in NPSLE are still very few and limited to patients with acute focal symptoms [19], [20]. In order
Quantitative MRI neuroimaging
In the last decade there are no doubt that modern quantitative neuroimaging techniques have represented the most impressive advance in understanding brain pathology in diffuse NPSLE. These techniques depend on different physical and chemical phenomena thus providing complementary information.
What should be done in daily clinical practice to approach SLE patients with NP symptoms?
There is a lot of evidence that NP manifestations, in the setting of SLE, occur frequently (in up to 80% of patients); they can be related to the disease itself (primary NP lupus) or to complications of the disease or treatment (secondary NP lupus). Conventional morphologic neuroimaging (i.e. MRI) cannot differentiate between past (chronic) and recent (acute) lesions. Because of the high probability of a NP event during the course of the disease, we think that it should be advisable to obtain a
Take-home messages
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Neuropsychiatric manifestations occur frequently in SLE and they can be related to the disease itself (primary NP lupus) or to complications of the disease or treatment (secondary NP lupus).
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Neuroimaging has greatly improved the understanding of NPSLE pathogenesis.
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None of the available techniques can actually be considered a “gold standard” for the diagnosis and follow up of NPSLE.
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For all different forms of NPSLE both a morphologic and a functional technique should be performed.
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Currently the
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