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Spinal cord compression by epidural lipomatosis in a patient with systemic lupus erythematosus.

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482
I . Youinou P, Moutsopoulos HM, Pennec YL: Clinical features of
SjBgren's syndrome. Curr Opin Rheumat01 2:687-692. 1990
2. Moutsopoulos HM, Youinou P: New developments in Sjogren's
9
1
syndrome. Curr Opin Rheumatol 3:815-822, 1
3. Chisholm DM, Mason DK: Labial salivary gland biopsy in
Sjogren's syndrome. J Clin Pathol 21:6S6-660, 1968
4. Greenspan JS, Daniels TE, Talal N , Sylvester RA: The histopathology of Sjogren's syndrome in labial salivary gland biopsies. Oral Pathol 37:217-229,1974
5 . Daniels TE, Silverman S Jr, Michalski JP. Greenspan JS,
Sylvester RA, Talal N : The oral component of Sjogren's syndrome. Oral Surg 39:875-885,1975
6. Daniels TE: Labial salivary gland biopsy in Sjogren's syn-
drome: assessment as a diagnostic criterion in 362 suspected
cases. Arthritis Rheum 27:147-156, 1984
7. Vitali C, Tavoni A, Simi U, Marchetti G, Vigorito P, d'Ascanio
A. Neri R, Cristofani R, Bombardieri S: Parotid sialography and
minor salivary gland biopsy in the diagnosis of Sjogren's syndrome: a comparative study of 84 patients. J Rheumatol 15:262267. 1988
8. Lindahl G, Lefvert AK. Hedfors E: Periductal lymphocytic
infiltrates in salivary glands in myasthenia gravis lacking Sjiigren's syndrome. Clin Exp lmmunol 66%-102. 19x6
9. Lindahl G . Hedfors E: Lymphocytic infiltrates and epithelial
HLA-DK expression in lip salivary glands in connective tissue
disease patients lacking sicca: a prospective study. Br J Kheumatol 28293-298. 1989
10. Manthorpe R, Oxholm P. Prause U. Schiodt M: The Copenhagen criteria for Sjdgren's syndrome. Scand J Kheumatol
SUPPI61:19-21,1986
Decreased hemoglobin levels in
nonsteroidal antiinflammatory drug-treated patients
may not be related to the drug
We recently reviewed the hemoglobin analyses from
several well-controlled double-blind studies in which rheumatoid arthritis patients were treated either with naproxen,
with a new antirheumatic drug (prinomide, CGS 10787B;
Ciba-Geigy, Summit, NJ), o r with placebo. Many people
believe that the use of nonsteroidal antiinflammatory drugs
(NSAIDs) is frequently associated with decreases in hemoglobin levels. However, in the studies we reviewed, it was
noted that some patients being treated with placebo, as well
as those treated with the active drug, had decreases in their
hemoglobin levels of 1-2 gm/dl over an 8-12-week period.
Table 1 shows the number of patients in each treatment group who had significant decreases (1-2 gm/dl) in their
hemoglobin values. As seen, 13.7% of the placebo-treated
patients had a significant decrease over a relatively short
period of time (4-12 weeks of treatment), compared with
6 2 2 % in the drug-treated groups.
Since reduction in the hemoglobin level is a common
finding in patients with rheumatic diseases. it is reasonable
to infer that an improvement in disease symptoms may be
reflected by an increase in the hemoglobin level and, conversely, that a decrease in hemoglobin level accompanies a
flare of symptoms. In an effort t o determine whether there
Table 1. Patients with decreases of 1-2 g d d l in the hemoglobin
(Hgb) level. by treatment group
No. (R) of
patients
Mean 2
SD decrease,
gddl
with de-
creased
Hgb
Treatment (n)
~~~~~~~~
~~~
Range of
decreases,
gddl
~
Prinomide. 1,500 &day (86) 18 (20.9) 1.42 2 0.26
Prinomide, 1,(K)0mg/day (46) 4 (8.7) I.23 2 0.32
3 (6.0) 1.42 2 0.46
Prinomide. 500 mg/day (50)
Naproxen. 750 mg/day (90) 20 (22.2) 1.3 2 0.27
18 (13.7) 1.33 2 0.24
Placebo ( I3 I )
1.0-1.7
1.&I.9
I.O-2.0
I.%I
.9
1.0-1.8
was ;I relationship between the decreases in hemoglobin
values and symptomatic changes in these patients. correlations between decreases in hemoglobin level and changes in
4 major efficacy parameters were determined. The efficacy
parameters chosen were investigator and patient assessment
of disease severity (measured on a 10-cm visual analog
scale), change in the number of tender joints (maximum 68).
and change in the number of swollen joints (maximum 66).
Using the Pearson product-moment method, no correlation
was found between decreases in hemoglobin level and
changes in any of these parameters. The amount of blood
drawn over the 8-12-week period of these studies (150-200
ml per patient) would not normally be expected to induce a
1-2 gm/dl decrease in the hemoglobin level.
These data indicate that significant decreases in the
hemoglobin level are not necessarily related to either NSAlD
use o r disease state, since nearly 14% of the placebo-treated
patients had significant decreases in their hemoglobin levels,
and there was no correlation with disease activity.
D. Innes Cargill, PhD
Tenere Consirlting
Tarrytown, N Y
Daniel E. Furst, M D
Robert Wood Johnson Medical School
N e w Brunswick, NJ
Spinal cord compression by epidural lipomatosis in a
patient with systemic lupus erythematosus
Epidural lipomatosis, a rare complication of systemic
glucocorticoid therapy, is characterized by abnormal accumulations of unencapsulated white fat on o r outside the
dura. Unlike excessive fat accumulations in other areas,
excessive deposition of fat in the epidural space may cause
serious morbidity, including complete paraplegia. In 1975,
Lee e t al reported the first case of epidural lipomatosis in a
renal transplant patient ( I ) . In other patients with this
CONCISE COMMUNICATIONS
condition, reported underlying medical problems have included heart transplant, polyarteritis nodosa, asthma,
Graves’ disease, lymphoma, hepatitis, rheumatoid arthritis,
juvenile rheumatoid arthritis, and dermatomyositis (2). Epidural lipomatosis has not previously been described as a
complication of systemic lupus erythematosus (SLE). We
describe herein an S L E patient in whom this condition
developed.
The patient was a 28-year-old, morbidly obese
woman with a 10-year history of steroid-dependent SLE.
Manifestations of lupus included a generalized seizure disorder, arthritis, facial rash, Raynaud’s phenomenon, myositis, pleurisy, peripheral neuropathy, and an affective disorder characterized by depression. She had been taking
prednisone at moderately high doses (>30 mg/day) for more
than 10 years. Immunotherapy with azathioprine and methotrexate had been initiated but was discontinued due to lack
of efficacy a n d o r toxicity. Four to five months prior to
admission, the patient developed back pain. One week prior
to our evaluation, she suddenly developed bilateral leg
weakness and urinary and fecal incontinence. Her prednisone dosage at the time of admission was 40 mg/day.
Examination revealed an obese woman with cushingoid features. Results of a general physical examination
483
were unremarkable except for the obesity and striae. Neurologic findings were normal except in her legs. Motor
strength testing revealed 115 strength in the right hip flexors
and 215 on the left. The knee flexor and extensor strength
was 015 bilaterally. Ankle dorsiflexion and plantar flexion
strength was 015 on the right and 115 and 215, respectively, on
the left. Sensation to pin prick and light touch were diminished below the level of T8. Proprioception, vibratory sense,
and deep tendon reflexes were absent in the legs. The rectal
examination revealed flaccid tone.
Abnormal laboratory data included a white blood cell
count of 28,000/mm3, a Westergren erythrocyte sedimentation rate of 41 m d h o u r , and gamma glutamyl transferase
and lactate dehydrogenase levels of 569 unitslliter and 301
unitslliter, respectively. An anteroposterior radiograph of
her thoracic spine revealed a possible T5 fracture.
Magnetic resonance imaging of the thoracic and
lumbar spine demonstrated multiple old compression fractures of the lumbar vertebral bodies and a subacute compression fracture of TS, with associated hematoma (Figure
I). There was excessive epidural fat present in both the
lumbar and thoracic regions (Figure 1). The epidural fat in
the thoracic region occupied more than 50% of the spinal
B
Figure 1. A, Sagittal and B, axial images of the thoracolumbar spine.
Curved (white) arrows show subacute compression fracture of T5 with
associated hemorrhage. Straight (black) arrows show extensive intraspinal
fat. The hemorrhage, fracture, and epidural fat produce ventral compromise of the thecal sac.
A
CONCISE COMMUNICATIONS
484
canal. causing significant compromise of the thecal sac. A
diagnosis of spinal epidural lipomatosis was made.
It was believed that the compression fracture, hematoma, and lipomatosis were compressing the spinal cord
at T5. A neurosurgeon was consulted about possible spinal
cord decompression. Because of the long duration of symptoms, morbid obesity, extensive length of the fat deposit,
and the need for internal fixation, it was concluded that the
operative risks were prohibitive in this patient. Strict dietary
control and reduction of the steroid dosage were recommended.
Azathioprine was reinstituted at a dosage of 75 mg
once daily, and the prednisone dosage was decreased to 30
mg/day. After 6 months of intensive physical therapy, during
which time the prednisone dosage was further reduced to
17.5 mg/day, muscle strength testing revealed 2/5 strength in
the right hip flexors and 315 on the left. Knee extension was
4 6 bilaterally. Foot extensor strength was 4/5 on the right
and 515 on the left. The patient's weight had decreased to 230
pounds. She still had urinary incontinence but had bowel
control.
Epidural lipomatosis, a condition characterized by
abnormal accumulations of unencapsulated fat on or outside
the dura, is extremely rare. This medical complication of
regular steroid use is unpredictable, but appears to be related
more to the duration of steroid administration than to
dosage. The presence of excessive fat in the spinal canal may
compromise the thecal sac to such an extent that a relatively
minor insult, such as the compression fracture in the present
case. causes symptoms. Fat deposition occurs most commonly in the thoracic region, but involvement of the lumbar
and sacral areas has been described. This diagnosis should
be considered in any patient receiving corticosteroids who
presents with signs or symptoms of spinal cord or cauda
equina compression. Differential diagnoses of these signs
and symptoms include vertebral fractures, hematoma, epidural abscess, transverse myelitis, arteriovenous malformation, congenital spinal stenosis, and malignancy, any of
which may be present in addition to epidural lipomatosis (2).
Myelography, though sensitive, cannot be used to
make a specific diagnosis. The diagnosis is best made by
computed tomography because fat has a low specific density
(-80 to -120 Hounsfield units) (3).
Surgical decompression is often indicated for treatment of epidural lipomatosis, and it can result in either rapid
relief of symptoms or slow recovery over months. Immediate decompressive laminectomy should be considered if
there is severe, acute compression with progressive neurologic signs. Conservative treatment with steroid reduction
and dietary restriction for weight loss have been variably
successful, especially in patients whose symptoms are mild
or whose general condition precludes surgical intervention (4).
Hulon E. Crayton, MD
Curtis R. Partington, MD
Carolyn L. Bell, MD
University of Wisconsin Hospital and Clinics
Modison, WI
I. Lee M, Lekias J , Gubbay SS, Hurst PE: Spinal cord compression
by extradural Fat after renal transplantation. Med J Ausl 1201203, 1975
2. Chapman PH, Martuza KL. Poletti CE, Karchmer AW: Lipomatosis associated with Cushing's syndrome. Neurosurgery 8:724727, 1981
3. Haid RW Jr, Kaufman HH. Schochet SS, Marano GD: Epidural
lipomatosis simulating an epidural abscess: case report and
literature review. Neurosurgery 2 I :744-747, 1987
4. George WE, Wilmot M. Greenhouse A, Hammeke M: Medical
management of steroid induced epidural lipomatosis. N Engl J
Med 308:31&319, 1983
Sweet's syndrome and monarthritis in a
human immunodeficiency virus-positive patient
Sweet's syndrome is an acute febrile neutrophilic
dermatosis, mostly affecting young adults ( I ) . It can be
associated with hematologic disorders, such as myeloid or
myelomonocytic leukemia. The exact etiology of Sweet's
syndrome is unknown, but a n infectious origin has been
suggested. We present herein the first report of Sweet's
syndrome occurring in association with human immunodeficiency virus (HIV) infection. The patient also had a monarthritis that was not typical of the arthritis often seen in
patients with Sweet's syndrome. The development of
Sweet's syndrome could be related to hypersensitivity to a
viral antigen.
The patient, a 30-year-old nurse, was admitted to the
hospital in July 1988 for evaluation of persistent swelling of
the left knee. Her clinical symptoms had begun 1 month
earlier, with the sudden onset of red lesions on the skin of
the face and the dorsal aspects of the hands and the wrists.
Sweet's syndrome was diagnosed on the basis of the clinical
findings. Prednisone (30 mg/day) was begun, and the symptoms improved rapidly. Ten days after admission, new skin
plaques appeared, and the patient reported having pain and
swelling of the left knee. Her prednisone dosage at that time
was 10 mg/day. The patient had a temperature of 38.2"C. and
swelling of the axillary lymph nodes was seen on physical
examination. A skin biopsy revealed perivascular and diffuse
dermal infiltration by polymorphonuclear cells, associated
with mild leukocytoclastic vasculitis (Figure 1).
The white blood cell count was 3.4 x 1Oy/liter,with
1,598 polymorphonuclear cells (PMN)/mm3 and 1,666 lymphocytes/mm'; the erythrocyte sedimentation rate was 62
mm/hour. Rheumatoid factor and antinuclear antibodies
were not found; anticardiolipin antibodies were detected
by enzyme-linked immunosorbent assay (ELISA; titer
> I :200). Tests for circulating immune complexes (polyethylene glycol-Clq) gave positive results. HIV was detected in
the serum by ELISA. and Western blot analysis showed
antibodies to gp160, gp141, pcore55, pcore40, ppo168, and
ppo134. The CD4 lymphocyte count was low (283/mm3); the
CD4:CD8 ratio was 0.25. HIV was not found in white cells
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spina, lupus, lipomatosis, epidural, patients, cord, systemic, compression, erythematosus
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