Systemic Lupus Erythematosus

KEY FACTS

TERMINOLOGY

• Systemic lupus erythematosus (SLE), neuropsychiatric

(NPSLE), CNS lupus

• Multisystem autoimmune disorder that affects respiratory,

cardiovascular, GI, GU, musculoskeletal systems, and CNS

○ CNS involved in up to 75%

IMAGING

• 4 general patterns

○ New infarcts (associated with ↑ anticardiolipin, ↑ lupus

anticoagulant antibodies)

○ Focal areas of hyperintensity, primarily in gray matter

○ Multiple T2WI hyperintensities (microinfarctions)

○ Extensive, reversible white matter (WM) changes

(cerebral edema)

• Multifocal WM microinfarcts, cerebral atrophy common

• Mild SLE: PET/SPECT more sensitive than MR

• Restricted diffusion (cytotoxic edema) in ischemia/infarct

• Increased diffusion (vasogenic edema) in vasculopathy

• Acute/active CNS lesions may enhance

TOP DIFFERENTIAL DIAGNOSES

• Multiple sclerosis (MS), Susac syndrome

• Lyme encephalopathy

• Arteriolosclerosis (microvascular disease)

• Other vasculitides (e.g., primary angiitis of CNS)

CLINICAL ISSUES

• Cerebral involvement may precede full-blown SLE picture

or may develop during course of disease

• SLE associated with antiphospholipid syndrome, posterior

reversible encephalopathy syndrome (PRES), Lupus-related

myelitis, Libman-Sacks endocarditis, emboli

DIAGNOSTIC CHECKLIST

• Look for elevated cerebrospinal fluid protein

• Role of imaging in SLE: Assess acute neurologic deficits

• Keep in mind: Negative brain MR does not exclude cerebral

lupus

(Left) Axial FLAIR MR in an SLE

patient with new neurologic

symptoms. Note the multiple

foci of FLAIR hyperintensity in

the white matter ﬇ and the

corpus callosum st. This

patient also had

antiphospholipid syndrome,

which is common in SLE

patients. (Right) Lateral

vertebrobasilar DSA shows

multifocal stenoses ﬈ typical

of a nonspecific vasculitis. This

is uncommonly seen with

lupus, which is more of a

small-vessel vasculitis than

other inflammatory

vasculitides. DSA is often

normal in SLE patients.

(Left) Axial FLAIR MR in a 52-

year-old woman with

neuropsychiatric symptoms

shows a large hyperintense

frontal lobe lesion ſt. (Right)

Coronal T1 C+ MR in the same

patient shows linear

enhancement throughout the

frontal lobe ﬇. Biopsy

disclosed CNS lupus vasculitis.

Although imaging of SLE is

nonspecific, there are often

multiple white matter

hyperintensities as well as

multifocal infarcts. Migratory

edematous regions are also

often seen.

Brain: Pathology-Based Diagnoses: Malformations,

Trauma, and Stroke

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