Lupus cerebritis: A case study

Kajs-Wyllie, Marylyn. Journal of Neuroscience Nursing34.4 (Aug 2002): 176-83.

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Nervous system involvement in systemic lupus erythematosus (SLE) occurs in 24%50% of all patients in the United States at some time during the course of their
illness. Lupus cerebritis with associated headache, seizures, stroke, and chorea is
just one of a wide array of central nervous system disorders SLE patients can
develop. It also is one of the most difficult manifestations of lupus to diagnose.
Advances in imaging and laboratory analysis have contributed to an earlier and
more specific diagnosis of lupus cerebritis. Despite improvements in the ability to
treat SLE, management of nervous system manifestations remains unsatisfactory.
Controversy exists as to the best approach for treatment. Newer combination
therapies based on anecdotal evidence are suggested.

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Headnote
Abstract: Nervous system involvement in systemic lupus erythematosus (SLE)
occurs in 24%-50% of all

Headnote
patients in the United States at some time during the course of their illness. Lupus
cerebritis with associated headache, seizures, stroke, and chorea is just one of a
wide array of central nervous system disorders SLE patients can develop. It also is

one of the most difficult manifestations of lupus to diagnose. Advances in imaging

and laboratory analysis have contributed to an earlier and more specific diagnosis
of lupus cerebritis. Despite improvements in the ability to treat SLE, management of
nervous system manifestations remains unsatisfactory. Controversy exists as to the
best approach for treatment. Newer combination therapies based on anecdotal
evidence are suggested.

Systemic lupus erythematosus (SLE) is a chronic, inflammatory disease of the

connective tissue. It is characterized by production of pathogenic autoantibodies
and immune complexes. Although SLE occurs in people of all ages and races and in
both genders, there is a higher incidence among females between 13 and 40 years
of age (Johnson, 1999). According to the American College of Rheumatology, for a
diagnosis of SLE, the patient must have at least four of the following organs
involved: renal (proteinuria or cellular casts in urine), cardiac (pleuritis/pericarditis),
skin (malar or discoid rash), joint (arthritis), hematologic system (anemia,
thrombocytopenia, neutropenia), or brain and spinal cord (seizure, psychosis,
myelitis; Johnson).

Because of its multisystem involvement, the neuroscience nurse may encounter the
SLE patient with central nervous system (CNS) manifestations. Kaposi in 1872
provided the earliest known report of the CNS effects of SLE with his description of
delirium in 2 of 11 SLE patients (Calabrese & Stem, 1995). Osler originally
postulated the cerebral vasculitis seen in SLE in 1903 when he described a patient
with lupus who also had neurological deficits (Liem, Gzesh, & Flanders, 1996).

More than 50% of all patients with SLE in the United States suffer from neurological
involvement (Bruyn, 1995; Moore, 1999). Kohen, Asherson, Gharavi, and Lahita
(1993) report that 25%-75% of SLE patients have neuropsychiatric manifestations at
some stage of their illness. Various mechanisms are postulated for the CNS
involvement found in SLE; diagnosis is difficult.

Controversy exists as to the best approach for treatment. The neuroscience nurse
may be challenged with the patient with cerebral lupus, who not only waits for the
definitive diagnosis but also hopes for response to treatment. This article discusses
the pathophysiology and proposed mechanisms of neurological involvement seen in
SLE. A case study is used to discuss treatment modalities and nursing interventions
for the patient with lupus cerebritis.

Mechanisms of CNS Involvement in SLE

To better understand the pathophysiology of lupus cerebritis, a review of the

vasculopathy seen in SLE is necessary. Lupus begins with the failure of T-helper cells
to moderate the immune reaction. The T-helpers, macrophages, and B cells,
mediated by cytokines (polypeptide products of activated lymphocytes), learn to
recognize antigens on cell surfaces. Immunoglobulins released by the B cells attach
to these antigens, forming immune complexes that collect in the tissues. Cytokine
release also triggers edema, thickening of the basement membrane, infiltration by
neutrophils, and disruption of the endothelium lining of the cell. This inflammation
and endothelial thickening causes vascular occlusion from an accumulation of fibrin
and thrombi, resulting in micro infarcts in various organs and, hence, SLE symptoms
(Johnson, 1999; Tsokos, 2001).

Knowledge about CNS involvement in SLE patients is based on work with mice as
well as on studies of postmortem human brains. The exact pathophysiological
process of lupus cerebritis is unknown. The proposed mechanisms (Table 1) are
likely due to the assault of several autoimmune system changes, including the
following (Bruyn, 1995; Howser, 1996):

* Circulating immune complexes

* Anti-neuronal antibodies

* Antiphospholipid antibodies

* Cytokine release.

Circulating Immune Complexes

The immune complexes, which consist of DNA and anti-DNA, cause an inflammatory
response as well as a disruption of the blood-brain barrier. These circulating
complexes have been found trapped in the highly vascular choroid plexus of SLE

patients upon autopsy. True vasculitis, however, is found only in about 10% of
patients with cerebral lupus (Bruyn, 1995; Liem et al., 1996).

Anti-neuronal Antibodies

The three identified anti-neuronal antibodies postulated in CNS involvement are the
lympho-cytotoxic antibodies (LCAs), which somehow react with brain tissue and
interfere with the neuron's ability to respond. The exact mechanism, however, is
unknown. LCAs have a specific role and are found in both the serum and
cerebrospinal fluid (CSF) of lupus patients with cerebritis. These antibodies also
correlate with cognitive and visual spatial defects. Second, the anti-neuronal
membrane antibodies are targeted directly to neuronal antigens. They, too, are
found in the serum of SLE patients with cerebritis. And third, the intracytoplasmic
antibodies target the constituents of the neuron cells (i.e., ribosomes and
neurofilaments). They are also called anti-SSA or anti-SSB antibodies and are found
in the CSF and serum. These antibodies are seen in 90% of SLE patients with
psychosis (Bruyn, 1995). Recently, neurotoxic metabolites have been found in the
CSF of mice that were behaviorally impaired from lupus-like autoimmune disease
(Marie et al., 2001).

Antiphospholipid Antibodies

Another mechanism of CNS involvement is the thrombosis associated with

antiphospholipid antibodies. The two antibodies implicated are anticardiolipin and
lupus anticoagulant. Anticardiolipin antibodies attach to the endothelial lining of
cells, causing endothelial damage, platelet aggregation, inflammation, and fibrosis.
The lupus anticoagulant antibody prolongs coagulation. Together the antibody
responses are known as the antiphospholipid antibody syndrome (Bruyn, 1995).
Stroke-like disorders, such as pulmonary emboli, miscarriage, thrombocytopenia,
and arterial/venous thrombi, are seen in 30%-50% of SLE patients (Terregino, 1999).

Cytokine Release

The final mechanism of lupus cerebritis involves the cytokines. The cytokines trigger
edema, endothelial thickening, and infiltration of neutrophils in brain tissue. Two
cytokines, interferon alfa and interleukin-6, have been found in the CSF of SLE

patients with psychosis. Research points to the possibility of an increased

concentration of these cytokines during SLE exacerbations (Bruyn, 1995).

No one clear mechanism appears to cause lupus cerebritis. All mechanisms may be
present or act independently.

Clinical Presentation

Lupus cerebritis may present as seizures, psychosis, myelopathy, or stroke in a

patient with SLE (Barr & Merchut, 1992). In its broadest definition, it is the
inflammatory response of the CNS secondary to SLE. A neurological disorder in SLE
may occur as an isolated event or in association with other systemic signs of SLE or
even precede the onset of systemic disease. Multiple neurological events may also
occur together (Khamashta, Cervera, & Hughes, 1991). CNS manifestations of SLE
are based on clinical studies, autopsies, or anecdotal reports of patients followed for
variable time periods.

Lupus cerebritis may occur in adults and children (Quintero-Del-Rio & Van, 2000;
Steinlin et al., 1995). The duration of the CNS involvement may vary from a few
minutes, as in classic migraine or a transient ischemic attack (TIA), to years, as in
dementia. Resulting neurological deficits may be transient or permanent,
occasionally resulting in death (Khamashta et al., 1991).

Focal Manifestations

Neurological signs are categorized into focal, nonspecific, and neuropsychiatric

(Table 2). Focal neurological signs include stroke, transverse myelitis, cranial nerve
palsies, peripheral neuropathy, and chorea, cerebellar ataxia (Barr & Merchut, 1992;
Calabrese & Stem, 1995). Infarction, both large vessel and microscopic, tends to
occur in isolation from other neurological events. The incidence of stroke is 3%-20%
of SLE patients. It is highest in the first 5 years of the disease and is directly
correlated with the presence of antiphospholipid antibodies (Terregino, 1999). The
stroke recurrence rate reported in the literature ranges from 13%-69% (Mitsias &
Levine, 1994; Bruyn, 1995).

Transverse myelitis occurs from demyelination or vasculopathy; small arteries are

often affected. There are reports in the literature of spinal cord infarcts and subdural
hematomas, resulting in paraplegia, sphincter dysfunction, and sensory loss (Moore,
1999). Cranial nerve palsies occur in 10%-15% of SLE patients. Laryngeal palsy,
visual loss, ptosis, and facial weakness are the more common manifestations (Barr
& Merchut, 1992).

Peripheral neuropathy occurs in more than 20% of the SLE population. This may
occur as carpal tunnel syndrome, numbness/tingling, facial pain, and ringing in the
ears (Moore, 1999). Movement disorders, such as cerebellar ataxia and chorea, are
seen in less than 5% of SLE patients (Barr & Merchut, 1992).

Nonspecific Manifestations

Nonspecific neurological signs occur in about 40%-70% of SLE patients. These

include headache, seizures, and organic brain syndrome. A "lupus headache" is the
most frequent manifestation of SLE. It can be intractable and of vascular or
muscular origin (Moore, 1999). If headache persists, cerebral venous thrombosis
must be considered. Although 40%-70% of lupus patients complain of headache, a
direct relationship with lupus and the severity of the disease is not always clear
(Barr & Merchut, 1992). Seizures occur in 20% of patients. Various types are
reported; tonic-clonic is most common (Terregino, 1999). These seizures are caused
by micro infarcts or subarachnoid hemorrhage (Barr & Merchut, 1992). The biggest
challenge in dealing with seizures and lupus is that so many medications used to
treat lupus also can cause seizures (e.g., steroids, antimalarials, and some
cytotoxics; Moore, 1999). Also, seizure medications may adversely affect SLE.
Valproate, in fact, may actually trigger the onset or exacerbation of lupus in some
predisposed patients (Barr & Merchut). Organic brain syndrome occurs in about 30%
of SLE patients due to multi-infarct dementia (Moore).

Neuropsychiatric Manifestations

The neuropsychiatric sequelae seen in SLE patients range from affective to

behavioral and cognitive disorders (Calabrese & Stem, 1995). Approximately 20% of

all lupus patients initially present with neuropsychiatric disorders (Wolf,

Niedermauer, Bergner, & Lowitzsch, 2001). Patients with undiagnosed lupus
cerebritis many times appear in psychiatric or neurologic clinics. Affective
symptoms include personality disorders, irritability, anger, anxiety, depression,
sadness, and feelings of hopelessness (Calabrese & Stem).

Behaviorally, SLE patients have episodes of emotional liability such as crying and
apathy, poor eye contact, and lack of initiative. Cognitive deficits are seen in
20%40% of SLE patients (Moore, 1999). Symptoms include difficulty in thinking,
concentrating, and speaking, with fluctuating levels of consciousness. Many patients
refer to this as "brain fog." SLE patients with these neuropsychiatric manifestations
are often referred for psychiatric consultation (Moore).

Psychosis may occur in SLE. However, the cause of psychosis is controversial as

CNS involvement from both SLE and steroid treatment can occur. Because steroids
are the mainstay treatment for SLE, it may be difficult to differentiate between
steroid psychosis or actual CNS involvement. West (1994) suggested that the best
way to differentiate between the two is to taper the steroid dose to determine
whether the signs and symptoms diminish. If the psychotic symptoms decrease,
steroid intoxication should be considered.

Diagnosing Lupus Cerebritis

Precise diagnosis of CNS lupus is extremely difficult. There is no single diagnostic

gold standard. Hanly (1998) recommended that diagnosis should be based on both
clinical assessment as well as the presence of antibodies in the serum and CSF
(Table 3).

Imaging Studies

A diagnosis of cerebral SLE cannot be made from radiologic findings alone, because
true cerebral vasculitis is rarely seen radiologically or even upon autopsy (Bruyn,
1995). Various imaging studies that aid in diagnosing lupus cerebritis have been
reported in the literature.

Computed tomography. Computed tomography (CT) scans may show a normal brain
or cerebral atrophy, calcification, infarcts, and intracranial hemorrhage or subdural
fluid collections (Calabrese & Stem, 1995; Raymond, Zariah, Samad, Chin, & Kong,
1996; Shaskey, Mijer, Williams, & Sawitzke, 1995). Some of these findings may be
attributed to chronic steroid use in the SLE patient.

Cerebral blood flow. Cerebral blood flow studies also can be used. One study
showed that patients with a long history of SLE frequently had decreased cerebral
blood flow (Postiglione et al., 1998).

Magnetic resonance imaging. Magnetic resonance imaging is considered a more

sensitive diagnostic tool for lupus cerebritis (Bruyn, 1995). In fact, 80%-100% of
those SLE patients who presented with seizures showed some type of an abnormal
MRI scan. MRI relaxometry, which segments gray matter, can pick up cerebral
edema from the cerebritis in specific locations (Petropoulos, Sibbitt, & Brooks,
1999). Diffusion-weighted, echo-planar MRI scans show primarily patterns of acute
and subacute infarction and vasogenic edema (Moritani et al., 2001). MRI
spectroscopy has recently been used, because it determines the presence of
neurochemical abnormalities and neurometabolite markers, which indicate cellular
damage. Those patients with suspected lupus cerebritis are shown to have positive
Nacetylaspartate, increased choline-compounds, lipids, and macromolecules, which
are all indicative of cell membrane breakdown and neuronal loss (Sabet, Sibbitt,
Stidley, Danska, & Brooks, 1998). Cerebral atrophy on MRI also has been correlated
with the presence of antiphospholipid antibodies (Hachulla et al., 1998).

Electroencephalography. Electroencephalography (EEG) also can be used to pinpoint

specific areas of damage from micro infarcts. EEG abnormalities are seen in 50%90% of SLE patients, including theta and delta slowing and sharp wave activity.
Evoked potentials can show auditory involvement (Calabrese & Stem, 1995; Glanz,
Schur, & Khoshbin, 1998).

Position emission tomography. Positron emission tomography (PET) has a sensitivity

of 90%. Patients with lupus cerebritis show abnormal cortical perfusion indicative of
cerebral hypometabolism. The use of PET and single photon emission computed
tomography (SPECT) scanning is controversial, however, because many medical
centers do not have this capability. It has been concluded that expensive PET and

SPECT scanning adds little information to the diagnosis of lupus cerebritis as

compared to MRI (Sailer et al., 1997; Waterloo et al., 2001).

Transcranial Doppler. Recently, transcranial doppler (TCD) testing was found to be a

noninvasive method to ascertain risk of stroke in patients with lupus cerebritis. The
presence of microthrombi can be seen (Kron, Hamper, & Petri, 2001).

Cerebral Angiogram. Even though the definitive method for diagnosis of cerebral
vasculitis is by cerebral angiogram, it is not recommended or routinely used. The
angiographic features can be nonspecific, because many times the vessels involved
are below the range of radiographic resolution (Liem et al., 1996). True vasculitis is
found in only about 10% of patients with cerebral lupus (Bruyn, 1995).

Laboratory Analysis

Because there is no one specific laboratory test available to diagnose lupus

cerebritis, diagnosing the condition remains a challenge (Bruyn, 1995). CSF studies
may be used, because they show high protein levels in 40%-80% of patients with
CNS manifestations of SLE (Calabrese & Stem, 1995). CSF also can be tested for the
presence of interleukin-6 and interferon alfa (cytokines), because their levels are
found to be significantly higher in SLE patients who develop neurological symptoms
(Gilad, Lampl, Eshel, Barak, & Sarova-Pinhas, 1997). In a study by Brundin et al.
(1998), which looked at the CSF of SLE patients with cerebritis, elevated levels of
nitric oxide were seen. These elevated levels were associated with more severe
neurological deficits. The author concluded that the presence of nitrates/nitrites in
CSF could be used to monitor activity or progression of the cerebritis.

Ten of the 19 different nuclear antigens are specific to SLE. The presence of
antinuclear antibodies (ANA) in the serum is used in the diagnosis of SLE. The
presence of DNA, anti DNA is the most specific test in 40%-60% of SLE patients. If
the ANA titer ratio is >1:64, a diagnosis of SLE is confirmed (Calabrese & Stem,
1995). Specific antibodies that target parts of the neuron and confirm CNS
involvement are intracytoplasmic targeted antibodies (anti-ribosomal P, anti Ro, SSA or anti-La, SS-B). Their presence is seen in both the CSF and serum of patients
with cerebritis (Bruyn, 1995).

The presence of antiphospholipid antibodies, lupus anticoagulant and

anticardiolipin, correlates with changes in the patient's CT/MRI. In a review of the
literature of more than 1,000 SLE patients with cerebritis, lupus anticoagulant was
seen in the serum of 34% of patients and anticardiolipin antibodies (i.e., IGG, IGA,
IGM) were seen in 44%-50% of patients (Mitchell, Webb, Hughes, Malsey, &
Cameron, 1994).

Neuron reactive autoantibodies are considered a much better marker for CNS
involvement, with levels significantly higher in SLE patients with cerebritis (Ochola,
Hussain, Khamashta, Hughes, & Vergani, 1995). Specifically, lympho-cytotoxic
antibodies (LCAs) are seen in 80% of patients (Bruyn, 1995). In general,
determination of an immunologic marker in the CSF is a better indicator of CNS
activity than the similar test in the serum (Barr & Merchut, 1992). Assessment of
complement components (C3 and C4), which are part of the coagulation cascade,
show low serum and CSF concentrations (Johnson, 1999).

Finally, diagnostic indicators for lupus cerebritis may include serial cognitive and
neuropsychological testing, which address motor function, dexterity, and verbal and
nonverbal abilities. In one study of patients who presented with neuropsychiatric
manifestations, 87% had abnormal tests in the Halstead-Reitan Neuropsychological
Test Battery and the Luria-Nebraska Battery (Barr & Merchut, 1992). One study
illustrated the benefits of using the Beck Depression Inventory in assessing
depression in patients with SLE (Iverson, Sawyer, McCracken, & Kozora, 2001).

Management of the Patient with Lupus Cerebritis: A Case Study

Treatment of lupus cerebritis is discussed by using a case study of a young female

who presented with symptoms of CNS involvement before even knowing she had
lupus.

History

Yvonne is a previously healthy 21-year-old female who just moved in with her
boyfriend. According to the patient, she and her boyfriend had been fighting
frequently because of her inability to search for a job due to weakness and fatigue.
Yvonne presented to the emergency department with multisystem involvement. She

complained of chills, a nonproductive cough, a fever of 101.6 deg F, diarrhea, and

increased muscle pain in her thighs, arms, chest, and back. She also reported
bleeding from her nose and vomiting and stated that her urine had looked "rusty
colored" over the past week.

Diagnosis Process

Admission labs showed renal and liver involvement with elevated blood urea
nitrogen and creatinine levels, and elevated liver function tests (LITEs). Urinalysis
was positive for protein, blood, and casts. Yvonne was admitted to the medical floor
for further workup. Intravenous (IV) fluids were started, an anti-emetic was given for
her nausea, and a cooling blanket for her fever. An abdominal ultrasound ruled out
obstruction. On day 2 of hospitalization, Yvonne had mental status changes, which
included uncontrollable crying, agitation, and repeated attempts to disrobe herself.
Her sister was greatly disturbed and was requesting a psychiatrist see the patient.
At this time the family was given help to cope with the sudden behavior changes of
the patient. They were taught how to interpret the behavior changes and how to
react to Yvonne's emotional outbursts by sitting quietly and keeping their voices
calm and quiet. It was especially difficult for the family, because there was still not a
definitive diagnosis.

A neurology consultation was obtained and an EEG ordered. The EEG showed
abnormal, persistent, diffuse, slow activity, but no seizures. An MRI scan with
magnetic resonance angiography ruled out any lesions or vasculitis. A lumbar
puncture, done to rule out infection, was normal. Yvonne was transferred to the
neurological intensive care unit for closer observation. She continued to complain of
muscle weakness and hip pain, necessitating TV narcotic analgesic. An infectious
disease consult was obtained to rule out an infectious cause for the myositis. Viral
serologies (Epstein Barr, IgG, IgM, HIV) were all negative. The elevated LFTs were
considered to be autoimmune in origin. A rheumatology consult was obtained,
because it was believed that the patient's clinical presentation could be consistent
with an autoimmune disorder. An antigen/antibody survey showed a positive ANA
with a titer of 1:1,600 (normal 1:64); SS-A and SS-B antigens were also identified.

Lupus anticoagulant/anticardiolipin antibodies were negative. She had low

complement levels: a C3 of 67 (normal = 83-184), and a C4 of 8 (normal = 17-59).
Based on the laboratory data and clinical picture, Yvonne was diagnosed with SLE,
and her altered mental status was thought to be secondary to CNS involvement.

Even though no one laboratory test confirmed cerebritis, the presence of the mental
confusion and the confirmed diagnosis of lupus supported the diagnosis of lupus
cerebritis.

After helping the family get through the many days of diagnostic testing (many of
the antibody tests had to be sent out for confirmation), the next step was deciding
on treatment options for Yvonne.

Treatment Modalities

The three main treatment options presented to the family included the use of
immunosuppressants, steroids, and anticoagulants. Immunosuppressive agents
such as cyclophosphamide and azathioprine are used to suppress circulating B and
T cells and autoantibody formation (Bruyn, 1995). These agents are usually given
intravenously as a bolus on a monthly basis. Steroids are used to reduce
inflammation and the immune response (Bruyn, 1995). Some advocate "large pulse
steroids" of TV methylprednisolone given over 20-30 minutes, 1.5 g every day for 3
days. After the IV dose, a low oral dose is given and then eventually tapered off.
High-dose steroids are indicated in cases of coma, seizures, psychosis, or transverse
myelitis (Bruyn, 1995). A favorable response to IV methylprednisolone and
cyclophosphamide has been seen in children with severe neuropsychiatric lupus
(Baca et al., 1999). Intrathecal injection of methotrexate plus dexamethasone is a
promising new method for treating CNS lupus (Dong et al., 2001).

Anticoagulation, with low-dose aspirin, warfarin, or subcutaneous or IV heparin are

possible third treatment options (Bruyn, 1995). Anticoagulation is started
prophylactically only if the patient suffers from thrombosis. Low-molecular-weight
heparinoids (LMWH) such as enoxaparin sodium, 30 mg every 12 hours
subcutaneously, has recently been studied and is considered the most effective
therapy for lupus patients with vascular thrombosis (Bick, 2001).

Psychotropic medications also are considered in certain patients. Antipsychotics are

given for acute psychosis, and antidepressants or benzodiazepines are given as
mood stabilizers or as adjunctive therapy when the patients are receiving steroids
(Calabrese & Stem, 1995). Part of the treatment regimen for some of the
neuropsychiatric manifestations such as major depression may include supportive
psychotherapy and electroconvulsive therapy (Calabrese & Stem, 1995).

Experimental therapies seen in the literature include five to seven exchanges of CSF
pheresis over 7-10 days. This provides additive and synergistic immunomodulation
(Barr & Merchut, 1992). Low doses of methotrexate, IV immunoglobulin (IgG), and
total lymphoid irradiation are treatment options that also help modulate the
overactive immune system (Bruyn, 1995; Sherer et al., 1999). A new
immunomodulating hormone therapy, a synthetic form of dehydroepiandrosterone
(prasterone) given orally, 200 mg a day, is being used to help reduce the need for
steroid treatment among patients with mild to moderate disease. Biologic agents
appear to be on the horizon for more severe SLE (Wallace, 2001).

Results

Yvonne was started on methylprednisolone 125 mg IV every 6 hours. Her mental

status continued to deteriorate over the next several days. She went from being
awake and alert, to being confused, and then localizing to pain. The steroid was
increased to 130 mg every 6 hours. On hospital day three, Yvonne's left upper
extremity was noted to be edematous and tender to touch. An ultrasound revealed
a thrombosis of her left subclavian vein. She was started on 1,000 units an hour of
IV heparin and eventually transitioned to warfarin, 10 mg orally. Her
hypercoagulable state was thought to be caused by her SLE.

On day four of her hospitalization, Yvonne desaturated to a pO^sub 2^ of 85%;

arterial blood gases showed an arterial pO^sub 2^ of 48. She did not require
intubation, but she was placed on a 100% nonrebreather mask. Pulmonary
embolism was being considered; a ventilation perfusion scan showed low
probability. The chest X ray, however, showed bibasilar infiltrates in the posterior
aspects of both lungs, consistent with aspiration. A pulmonary consult was obtained
and Yvonne was placed on antibiotics. A central-line catheter was placed for total
parenteral nutrition (TPN). During this time the family was kept informed of all the
changes in her condition because of the multisystem involvement of her SLE. The
social worker and chaplain were helping them cope with the changes that seemed
to be happening daily.

The next day the nurses noted that Yvonne's middle digit on her left hand was
bluish in color. The rheumatologist determined that this was due to her continuing
hypercoagulable state. Her steroids were increased to a pulse dose of 500 mg over
30 minutes for 3 days and then returned to 125 mg every 6 hours. A dose of TV
cyclophosphamide, 500 mg, was given for immunosuppression.

After 7 days on steroids, Yvonne's mental status improved. She responded to voice
and became more alert and oriented. Now that she was more cooperative, physical
and occupational therapy began for more active range of motion, muscle
strengthening, and assistance with activities of daily living. Yvonne was anemic
throughout her hospitalization and received a total of 4 units of packed red blood
cells. She also was thrombocytopenic and neutropenic, which was attributed to both
her heparin and the dose of cyclophosphamide. She was given filgrastim, 250 mcg a
day subcutaneously, for 4 days.

Yvonne continued to show progress. By day 12 of her hospitalization, she was

weaned to room air and was transferred to the neurological floor. A swallow
evaluation was done and trial feedings were begun. She eventually progressed to a
mechanical soft diet without supervision; the TPN and central line were
discontinued. A cognitive evaluation and treatment program were begun for
difficulties Yvonne had with processing. A family care conference was held to plan
discharge needs. The family was given the name of a contact person for the Lupus
Support Group. On day 15 of her hospitalization, Yvonne was ready to transfer to
her father's care and follow-up at a clinic near her father's home. Her steroids were
being tapered as followed: prednisone 25 mg orally twice a day for 8 days, then 20
mg orally twice a day. Other medications she was continued on included warfarin,
7.5 mg, to be adjusted as necessary to maintain an INR goal of 2-3.

According to the literature, CNS involvement ranks second to renal failure as cause
of death in the patient with SLE (Barr & Merchut, 1992). Prognosis is guarded
because the multisystem involvement of lupus can continue with exacerbations and
remissions. Yvonne survived 16 days of hospitalization with a new diagnosis of
lupus. She encountered various infections and problems from her hypercoagulable
state, including CNS involvement. She left the hospital with concentration and
memory deficits. It was strongly recommended she seek continued cognitive
therapy at her hometown clinic. Yvonne continues living with her father. She ended
her relationship with her boyfriend and is employed at a fast food restaurant. One
month after discharge from the hospital, she was still on low-dose steroids and
received one additional dose of cyclophosphamide.

Summary

Lupus cerebritis may be the first indication of SLE. No one diagnostic test is
conclusive of the CNS involvement in SLE; however, clinical manifestations support
diagnosis. Various treatment options are utilized to aid with immunosuppression and
the hypercoagulable state. The CNS involvement seen in lupus cerebritis is difficult
for the family to deal with not only because of the sudden changes, but also
because the waiting period for exact diagnosis may lead the family into a very
difficult coping period. The neuroscience nurse has an important role in
understanding not only the CNS involvement of SLE but also its diagnostic and
treatment modalities, to help the patient and family cope with this sometimes
devastating disease.

Sidebar
Intrathecal injection of methotrexate plus dexamethasone is a promising new
method for treating CNS lupus.

Sidebar
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References
References

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Barr, W.G., & Merchut, M.P. (1992). Systemic lupus erythematosus with central
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Bick, R.L. (2001). Antiphospholipid thrombosis syndromes. Clinical Applications of

Thrombosis and Hemostasis, 7, 241-258.

Brundin, L., Svenungsson, E., Morcos, E., Andersson, M., Olsson, T., Lundberg, I., et
al. (1998). Central nervous system nitric oxide formation in cerebral systemic lupus
erythematosus. Annals of Neurology, 44, 704-706.

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AuthorAffiliation
Questions or comments about this article may be directed to: Marylyn Kajs-Wyllie,
MSN RN CCRN CNS, St, David's Medical Center, 919 E. 32nd Street, Austin, TX
78705. She is a neuroscience clinical nurse specialist at the Neuroscience Center at
St. David's.

Copyright American Association of Neurosurgical Nurses Aug 2002