Case Report
Myelitis Complicating Systemic Lupus
Erythematosus: Successfully Treated with
Corticosteroids and Cyclophosphamide
Wafa Ammouri, MD, Zoubida Tazi Mezalek, MD, PhD, Hicham Harmouche,
Mohammed Aouni, MD, PhD, Mohammed Adnaoui, MD, PhD,
and Abdelaaziz Maaouni, MD, PhD
Abstract: Systemic lupus erythematosus (SLE), a multisystem au-
toimmune disease with protean manifestations, occurs with neuro-
psychiatric manifestations in ⱕ60% of patients. Myelitis is a rare but
serious complication of SLE. The diagnosis of myelitis may be
difficult, but magnetic resonance imaging is generally very useful in
assessing the extension and severity of lupus-related myelitis. We
present the case of a 22-year-old woman with SLE since age 18 who
presented with bilateral leg paresis of 7 days’ duration.
Key Words: corticosteroids, myelitis, systemic lupus erythematosus
S ystemic lupus erythematosus (SLE) is a multisystem auto-
immune disease with protean manifestations. Neuropsychi-
atric manifestations are present in ⱕ60% of patients with SLE
with psychosis, seizures, headaches, aseptic meningitis, and ce-
rebrovascular accidents.1,2 Myelitis is a rare but serious compli-
cation of SLE. Previous reports indicate a prevalence of approx-
imately 1–2%.1,3 The diagnosis of myelitis may be difficult, but
magnetic resonance imaging (MRI) is generally very useful in
assessing the extension and severity of lupus-related myelitis.4
Case Reports
Case 1
A 22-year-old woman with SLE since age 18 pre-
sented with bilateral leg paresis of 7 days’ duration. On
admission, her body temperature was 37°, heart rate was
From the Department of Internal Medicine, Ibn Sina Hospital, Rabat,
Morocco.
Reprint requests to Wafa Ammouri, MD, Department of Internal Medicine,
Ibn Sina Hospital, 29, OLM, Souissi II, Rabat, Morocco 10000. Email:
wafaammouri@hotmail.com
Accepted February 17, 2009.
Copyright © 2009 by The Southern Medical Association
0038-4348/0⫺2000/10200-0744
744
MD, PhD,
70 beats/min, and her blood pressure was 130/60 mm Hg.
Neurological examination showed spastic paraparesis; deep
tendon reflexes of the lower extremities were lively. Bab-
inski reflex was bilaterally positive. Laboratory findings
were: erythrocyte sedimentation rate 60 mm/h; white blood
cell count 3200/mm3; lymphocytes 800/mm3; and hemo-
globin level 9 g/dL. Serological examination showed hy-
pocomplementemia (C4 of 5 mg/dL, C3 of 30 mg/dL), pos-
itive antinuclear, anti-DNA, and anticardiolipin antibodies.
MRI of the spinal cord revealed increased signal
intensity in T2-weighted images of the medulla oblon-
gata and the thoracic spine (Fig. A). Cerebrospinal fluid
analysis showed no abnormality. She was diagnosed with
transverse myelitis with antiphospholipid antibodies; she
was treated with high-dose corticosteroids, anticoagulant
therapy, and cyclophosphamide pulses. Azathioprine was
introduced after 12 pulses of cyclophosphamide. After
one year of follow up, she remains asymptomatic.
Case 2
A 22-year-old woman with SLE since age 19 was
admitted for headaches, altered consciousness, and fever.
On admission, her body temperature was 39°, heart rate was
100 beats/min, and her blood pressure was 110/60 mm Hg.
(continued next page)
Key Points
• Myelitis is a rare and serious complication of systemic
lupus erythematosus (SLE).
• The pathogenesis of myelitis in SLE is uncertain, and
diagnosis is made by magnetic resonance imaging.
• The outcome of myelitis in SLE is variable. Early
management and aggressive treatment of patients are
necessary for a favorable response.
© 2009 Southern Medical Association

(Case Report continued from previous page)
General examination revealed anemic conjunctivae and
hair loss. Neurological findings revealed diplopia, nu-
chal stiffness, and paresthesias in all limbs without
sphincter incontinence. Deep tendon reflexes were
present. Babinski reflex was bilaterally negative. Labo-
ratory findings were: erythrocyte sedimentation rate 100
mm/h; white blood cell count 2200/mm3, lymphocytes
1000/mm3; and hemoglobin level 9.4 g/dL. Serological
examination showed hypocomplementemia (C4 of 4 mg/
dL, C3 of 20 mg/dL), positive antinuclear, anti-DNA
antibodies. Prothrombin time and activated partial throm-
boplastin time were normal. Lupus anticoagulant and
Discussion
The pathogenesis mechanism of myelitis in SLE is un-
certain, although vasculitis and arterial thrombosis resulting
in ischemic cord necrosis have been suggested.5
In our first case, lupus myelitis was associated with
the presence of antiphospholipid antibodies. In the litera-
ture, the prevalence of antiphospholipid antibodies has
been reported in over 60% of patients with lupus myelop-
athy, and it was implicated as having a pathogenic role.3
Fig. A, Sagittal T2-weighted magnetic resonance image of the
lumbar spine showing high signal intensity from level T1 to
T5. B, Sagittal T2-weighted magnetic resonance image of the
cervical spine showing high signal intensity from level C1
to C6.
Southern Medical Journal • Volume 102, Number 7, July 2009
Case Report
anticardiolipin-ß2GPI antibody were also negative. Ce-
rebrospinal fluid analysis showed pleocytosis, elevated
protein, and normal glucose concentration. Gram stain
and cultures were negative. Brain and cervical MRI re-
vealed cerebral vasculitis with high-intensity signal of
the spinal cord (Fig. B). Despite methylprednisolone
pulses, three days later she developed ischemic cerebral
stroke (hemiparesis). Subsequent therapy with cyclo-
phosphamide pulses and anticoagulant resulted in a com-
plete recovery. Mycophenolate mofetil was introduced
after 6 pulses of cyclophosphamide. After two years, she
remains well.
There have been conflicting data in the literature about the
timing of myelitis compared with the onset of SLE. In a
study of 14 cases of transverse myelopathy (TM) related to
SLE, Kovacs et al3 found that TM occurred as the initial
manifestation of SLE in 39% of patients; and in 42%, TM
presented within the first five years of diagnosis with SLE.
The prominent presentation of TM in SLE seems to be
sensory, predominantly in the thoracic area. The outcome
of myelitis in SLE was variable between the cases re-
ported, and the morbidity rate was 50%.6
There are no established guidelines for the treatment
of myelitis in SLE.3,4,6 Regardless of the presence or ab-
sence of antiphospholipid antibodies, the optimal window
for treatment initiation is unclear. It has been postulated
that aggressive treatment within one week of the onset of
symptoms is crucial for a favorable response.6 Treatment
with methylprednisolone pulse followed by intravenous
cyclophosphamide seems to be most effective. Plasma-
pheresis also has been successfully performed in some
reports.3,4 Other modalities such as mycophenolate mofetil,
autologous stem cell transplantation, and intrathecal dexa-
methasone with or without methotrexate have been sug-
gested for treatment.6
References
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2. West SG. Lupus and central nervous system. Curr Opin Rheumatol 1996;
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3. Kovacs B, Lafferty LT, Brent LH, et al. Transverse myelopathy in sys-
temic lupus erythematosus: an analysis of 14 cases and review of the
litterature. Ann Rheum Dis 2000;59:120 –124.
4. Deodhar AA, Hochenedel T, Bennett RM. Longitudinal involvement of
the spinal cord in a patient with lupus related transverse myelitis. J Rheu-
matol 1999;26:446 – 449.
5. Shymalan NC, Singh SS, Bisht DB. Transverse myelitis after vaccination.
Br Med J 1964;1:434 – 435.
6. Lu X, Gu Y, Wang Y, et al. Prognostic factors of lupus myelopathy.
Lupus 2008;17:323–328.
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