Elsevier

Autoimmunity Reviews

Volume 7, Issue 4, February 2008, Pages 297-304
Autoimmunity Reviews

Optimizing clinical monitoring of central nervous system involvement in SLE

https://doi.org/10.1016/j.autrev.2007.11.022Get rights and content

Abstract

Central Nervous System (CNS) involvement is a frequent SLE manifestation occurring in 15–75% of patients. However, diagnosis of CNS involvement is a difficult task and requires a careful clinical and laboratory assessment along with instrumental evaluation. In recent years major advances in neuroimaging techniques allowed a great improvement in our understanding of SLE pathogenesis. Anyway, since no single imaging technique covers all brain pathology and both inflammation and neurodegeneration contribute to SLE pathogenesis, it is very important to use a multimodality approach coupling a morphological with a functional imaging modality. In this setting, to date, conventional magnetic resonance imaging and single photon emission computed tomography are the most largely available and accessible techniques. Modern techniques such as perfusion weighted imaging, diffusion weighted imaging, magnetization transfer imaging and magnetic resonance spectroscopy provide useful information to assess brain tissue damage however, their clinical relevance in individual patients needs further evidence.

In this review we would like to summarize what have we learned in the last few years about neuroimaging in NPSLE, what have been major advances in neuroimaging techniques and, finally, we would like to give some suggestions about what should be done in daily clinical practice to approach SLE patients with NP symptoms.

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

  • 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).

  • Neuroimaging has greatly improved the understanding of NPSLE pathogenesis.

  • None of the available techniques can actually be considered a “gold standard” for the diagnosis and follow up of NPSLE.

  • For all different forms of NPSLE both a morphologic and a functional technique should be performed.

  • Currently the

References (40)

  • W.L. Sibbitt et al.

    Neuroimaging in neuropsychiatric systemic lupus erythematosus

    Arthritis Rheum

    (1999)
  • J.E. Jennings et al.

    Value of MRI of the brain in patients with systemic lupus erythematosus and neurologic disturbance

    Neuroradiology

    (2004)
  • T. Csepany et al.

    MRI findings in central nervous system systemic lupus erythematosus are associated with immunoserological parameters and hypertension

    J Neurol

    (2003)
  • C.A. Roldan et al.

    Valvular heart disease is associated with nonfocal neuropsychiatric systemic lupus erythematosus

    J Clin Rheumatol

    (2006)
  • M. Govoni et al.

    Recent advances and future perspective in neuroimaging in neuropsychiatric systemic lupus erythematosus

    Lupus

    (2004)
  • A. Otte et al.

    Brain glucose utilization in systemic lupus erythematosus with neuropsychiatric symptoms: a controlled positron emission tomography study

    Eur J Nucl Med

    (1997)
  • P. Colamussi et al.

    Brain single-photon-emission tomography with 99mTc-HMPAO in neuropsychiatric systemic lupus erythematosus: relations with EEG and MRI findings and clinical manifestations

    Eur J Nucl Med

    (1995)
  • W.J. Lin et al.

    Technetium 99-m-HMPAO brain SPECT in systemic lupus erythematosus with CNS involvement

    J Nucl Med

    (1997)
  • K. Oku et al.

    Cerebral imaging by magnetic resonance imaging and single photon emission computed tomography in systemic lupus erythematosus with central nervous system involvement

    Rheumatology

    (2003)
  • X. Zhang et al.

    Diagnostic value of single photon emission computed tomography in severe central nervous system involvement of Systemic lupus erythematosus: a case control study

    Arthritis Rheum

    (2005)
  • Cited by (36)

    • Neuroimaging findings in rheumatologic disorders

      2021, Journal of the Neurological Sciences
    • Central nervous system manifestation of lupus erythematosus resembling brain abscess

      2019, International Journal of Pediatrics and Adolescent Medicine
      Citation Excerpt :

      NAA is reduced in CNS lesions of patients with SLE, but it can also be marked in the normal-appearing brain. However, NAA changes are more profound in patients with NPSLE and can be permanent, which implies neuronal death or transitory damage to the cells [8,9]. Choline levels were shown to be elevated in patients with NPSLE, and its levels correlated with disease activity [10].

    • The diagnosis and clinical management of the neuropsychiatric manifestations of lupus

      2016, Journal of Autoimmunity
      Citation Excerpt :

      As a general rule it could be useful to couple a structural (i.e MRI) with a functional neuroimaging study such as SPECT. In combination with MRI, this approach has demonstrated to show a prevalent negative predictive value in ruling out NPSLE when both techniques are negative [99]. Other modalities (MRS, PET, PWI, etc.) are usually less available and less used in every day clinical practice.

    • A volcanic explosion of autoantibodies in systemic lupus erythematosus: A diversity of 180 different antibodies found in SLE patients

      2015, Autoimmunity Reviews
      Citation Excerpt :

      This observation raises the question of how these autoantibodies contribute to the pathogenesis of SLE. A wide variety of imaging techniques are increasingly used to diagnose CNS involvement including morphological modalities such as MDCT, MRI, MTI, and MRS or functional ones as PET, SPECT and fMRI [35–40]. While numerous studies have attempted to correlate serological profiles to radiological features [41,42], only few elicited such an association.

    View all citing articles on Scopus
    View full text