Table 4

Illustrative cases of SLE patients presenting with neuropsychiatric manifestations

Management based on EULAR recommendations
Case 1
 48-Year-old woman with SLE and history of single seizure; serology notable for anti-ds DNA (+), antiphospholipid (−)This patient has possible cognitive dysfunction. Risk factors include previous major NPSLE (seizure) and generalised SLE activity. Neuropsychological testing (1 h ACR battery) diagnosed moderate cognitive dysfunction. Brain MRI was performed due to the young age and the progressive cognitive decline reported by the patient. She received glucocorticoids and azathioprine due to generalised SLE activity and brain MRI lesions. Follow-up neuropsychological examination after 6 months.
 Presents with progressive memory decline and difficulties with speech and complex task completion
 Non-CNS SLEDAI: 8; Brain MRI: periventricular WM focal T2-weighted hyperintensities
Case 2
 38-Year-old woman with history of SLE nephritis (class III); serology: anti-dsDNA (+), antiphospholipid (−)This patient has ischaemic stroke. Brain MRI with DWI showed ischaemic lesions (left middle cerebral artery) and the MRA revealed thrombosis of the left internal carotid and the left middle cerebral arteries. Carotid artery ultrasound showed mild atherosclerotic lesions. The patient was started on aspirin and statin, and she was advised to stop smoking. Blood pressure was controlled by ACE-inhibitor. Disease activity was controlled with low-dose glucocorticoids and hydroxychloroquine.
 Smoker, dyslipidemia (LDL-C 150 mg/dl)
 Presents with right hemiparesis and motor aphasia
 Modest lupus activity (SLEDAI: 6)
Case 3
 16-Year-old woman presents with fever and generalised tonic-clonic seizures/status epilepticusThis patient has SLE with seizure disorder. Brain MRI was normal and the EEG revealed epileptiform activity. The patient received anti-epileptic therapy (phenobarbital), and glucocorticoids, hydroxychloroquine, and azathioprine to control disease activity. She was scheduled for a follow-up EEG to guide duration of AED therapy.
 CSF examination with mild pleocytosis and increased protein; negative gram stain and PCR for HSV
 Arthritis, malar rash, alopecia, lymphadenopathy
 Serology: ANA 1:640, anti-dsDNA (+), antiphospholipid (−)
Case 4
 31-Year-old woman with SLE. Major depression progressively deteriorating for the last year interfering with work. Mild SLE activity (malar rash, mild arthritis)This patient has mood disorder (major depression) with poor response to various antidepressants. Brain MRI—performed due to her severe, progressive symptoms—was normal. Disease activity was controlled with glucocorticoids and azathioprine with moderate improvement in her depression.
Case 5
 28-Year-old woman with SLE and several weeks of new-onset headaches of moderate severity. No focal neurological signs or fever. Clinical: arthritis, malar rashThis patient has headache with some concerning features (new-onset, persistent moderate severity). Brain MRI showed subcortical WM focal hyperintensities. The patient received short-course of low-dose glucocorticoids with clinical improvement.
 Now presents with tingling sensation in the hands in the absence of arthritis; antiphospholipid (−)Needle electromyography and NCS were unremarkable. Spinal cord MRI revealed longitudinal myelitis (C2–C7, T1). Mild CSF abnormalities (7 cells/hpf, protein 66 mg/dl, IgG index (−)). The patient received pulses IV-MP and immunosuppressive therapy.
Case 6
 23-Year-old man with SLE, mild arthralgias and a previous history of discoid rash and serositisThis patient has stroke in the context of SLE-related APS. Brain MRI revealed infarct in the right parietal lobe. Treated with oral anticoagulants (target INR 3.1–4). Low dose of glucocorticoids was continued with gradual tapering and hydroxychloroquine was added. After 3 years of follow-up, no new cerebrovascular events were reported.
 He presents with left hemiparesis. No history of hypertension or smoking.
 Serology: anti-DNA (−), C3, C4: normal, LAC: positive, aCL IgG: 55 GPL, no hyperlipidemia
  • aCL, anticardiolipin antibodies; AED, anti-epileptic drug; ANA, antinuclear antibody; APS, antiphospholipid syndrome; CSF, cerebrospinal fluid; CNS, central nervous system; DWI, diffusion-weighted imaging; EULAR, European League Against Rheumatism; GPL, IgG anticardiolipin unit (1 GPL units = 1 μg affinity-purified IgG ACA from an original index serum sample); HSV, herpes simplex virus; INR, international normalised ratio; IV-MP, intravenous methylprednisolone; LAC, lupus anticoagulant; LDL-C, low-density lipoprotein cholesterol; MRA, magnetic resonance angiography; NCS, nerve conduction studies; NPSLE, neuropsychiatric systemic lupus erythematosus; SLE, systemic lupus erythematosus; SLEDAI, SLE disease activity index; WM, white matter.