The n e w e ng l a n d j o u r na l of
Case Records of the Massachusetts General Hospital
From the Departments of Ophthalmology
(D.M.C., J.F.R.) and Radiology (M.E.C.),
Massachusetts Eye and Ear, the Depart‑
ment of Medicine, Massachusetts Gener‑
al Hospital (J.H.S.), and the Departments
of Ophthalmology (D.M.C., J.F.R.), Radi‑
ology (M.E.C.), and Medicine (J.H.S.),
Harvard Medical School — all in Boston.
N Engl J Med 2018;378:282-9.
DOI: 10.1056/NEJMcpc1701763
Copyright © 2018 Massachusetts Medical Society.
282
m e dic i n e
Founded by Richard C. Cabot
Eric S. Rosenberg, M.D., Editor
Virginia M. Pierce, M.D., David M. Dudzinski, M.D., Meridale V. Baggett, M.D.,
Dennis C. Sgroi, M.D., Jo‑Anne O. Shepard, M.D., Associate Editors
Allison R. Bond, M.D., Case Records Editorial Fellow
Emily K. McDonald, Sally H. Ebeling, Production Editors
Case 2-2018: A 41-Year-Old Woman
with Vision Disturbances and Headache
Dean M. Cestari, M.D., Mary E. Cunnane, M.D., Joseph F. Rizzo III, M.D.,
and John H. Stone, M.D., M.P.H.​​
Pr e sen tat ion of C a se
Dr. John H. Stone: A 41-year-old woman was evaluated in the rheumatology clinic of
this hospital because of headaches and a 6-week history of intermittent, transient
vision loss.
Four years before the current presentation, a skin eruption on the right foot and
a lesion on the tongue developed. A biopsy of both lesions was performed at an-
other hospital; examination of the specimens revealed evidence of leukocytoclastic
vasculitis. Prednisone, methotrexate, mycophenolate mofetil, and azathioprine
were administered, and the mucocutaneous lesions improved, but they recurred
after any attempt to taper the immunosuppressive medications.
Twenty-one months before the current presentation, headaches developed and
skin lesions recurred. The headaches were generally located in the right temporal
area, were characterized as throbbing, and lasted approximately 5 to 10 minutes.
The patient was evaluated at the other hospital, and magnetic resonance imaging
(MRI) of the head with contrast enhancement was performed.
Dr. Mary E. Cunnane: Axial, T2-weighted fluid-attenuated inversion recovery
(FLAIR) images showed hyperintensity in the right thalamocapsular region and
right midbrain, with patchy enhancement of the right midbrain (Fig. 1).
Dr. Stone: A lumbar puncture was performed. On cerebrospinal fluid (CSF)
analysis, the protein level was 54 mg per deciliter (reference range, 5 to 55) and
the total nucleated-cell count was 16 per cubic millimeter, with 66% lymphocytes.
The opening pressure was not measured. A test for cryptococcal antigen and
nucleic-acid tests for toxoplasma, JC virus, herpes simplex virus types I and II, and
varicella–zoster virus were negative. Flow cytometry did not show any abnormal
cell populations. Screening tests for human immunodeficiency virus and syphilis
were negative. A presumptive diagnosis of central nervous system (CNS) vasculitis
was made. Prednisone, cyclophosphamide, rituximab, and hydroxychloroquine
were administered, and the headaches improved temporarily. Two months later,
n engl j med 378;3 nejm.org  January 18, 2018
 Case Records of the Massachuset ts Gener al Hospital
A B
Figure 1. MRI of the Head Obtained 21 Months before Presentation.
Axial T2‑weighted fluid‑attenuated inversion recovery images show hyperintensity in the right thalamocapsular region
(Panel A, arrow) and right midbrain (Panel B, arrow), with patchy enhancement of the right midbrain (Panel C, arrow).
repeat MRI of the head reportedly showed reso-
lution of the hyperintensity that had been seen
previously.
Eighteen months before the current presenta-
tion, the patient was referred to the rheumatol-
ogy clinic at this hospital for the evaluation of
recurrent painful mucocutaneous lesions of the
hands, feet, and mouth. She also reported fa-
tigue and arthralgias of the hands, knees, and
ankles. Blood tests revealed antinuclear anti-
bodies (at a titer of >1:5120, with a speckled
pattern) and anti-Ro antibodies (154.60 optical-
density units; normal range, <19.99), as well as
hypocomplementemia. A diagnosis of systemic
lupus erythematosus (SLE) was suspected. Pred-
nisone and hydroxychloroquine were continued,
and mycophenolate mofetil was added to the
regimen. Cyclophosphamide and rituximab were
stopped. Over the next 15 months, the patient
had exacerbations of the cutaneous vasculitis
whenever the prednisone dose was tapered.
Three months before the current presenta-
tion, the prednisone dose was increased because
of worsening lesions on the hands and feet. Dur-
ing the 6 weeks before this presentation, spo-
radic episodes of transient vision loss occurred.
The episodes lasted 5 to 10 seconds, occurred one
to several times per day, and remitted spontane-
ously. They were associated with dizziness, pul-
satile tinnitus, and headaches. The headaches
reportedly worsened with changes in position,
such as bending over, but there appeared to be
no relationship between the headaches and time
of day, diet, activity, or performance of the Val-
salva maneuver.
n engl j med 378;3
C
The patient was evaluated by an ophthalmolo-
gist 1 week before the current presentation and
then evaluated by her neurologist at another facil-
ity 6 days later. On neurologic evaluation, the
blood pressure was 114/72 mm Hg. Bilateral
optic-disk swelling was noted, but the remainder
of the examination was reportedly normal. MRI
of the head was performed.
One day later, the patient was evaluated by
her rheumatologist at this hospital. She reported
ongoing mucocutaneous symptoms, including
painful swelling on the right lip, a painful ulcer
on the palate, and pruritic lesions on the feet.
There was no diplopia, neck stiffness, photopho-
bia, sonophobia, general or focal weakness, par-
esthesia, ataxia, syncope, fever, weight loss, nau-
sea, vomiting, or bowel or bladder dysfunction.
The patient’s medical history was notable for
weight gain of 22.7 kg over a 4-year period in
the context of glucocorticoid treatment and
glucocorticoid-related osteoporosis. Her medica-
tions included prednisone, hydroxychloroquine,
mycophenolate mofetil, alendronate, calcium, and
vitamin D. Rituximab had been readministered
9 days earlier.
The patient was originally from the eastern
Mediterranean and had immigrated to the United
States 20 years earlier. She was divorced and had
one adolescent son. She had previously worked
in the restaurant business but had to discontinue
this work because of physical limitations. She
had an 8.5-pack-year history of cigarette smok-
ing and continued to smoke several cigarettes a
day, but she did not drink alcohol or use illicit
drugs. Her mother had coronary artery disease,
nejm.org January 18, 2018 283
 The n e w e ng l a n d j o u r na l of m e dic i n e

Table 1. Laboratory Data.*

the pulse 109 beats per minute, the blood pressure
129/78 mm Hg, the respiratory rate 16 breaths per
Reference Range, 9 Days before minute, and the oxygen saturation 100% while
Variable Adults† Presentation
the patient was breathing ambient air. The weight
Sodium (mmol/liter) 135–145 142 was 94 kg. The patient appeared mildly unwell,
Potassium (mmol/liter) 3.4–5.0 3.7 was tearful, and had cushingoid features. The
Chloride (mmol/liter) 100–108 101 bilateral optic-disk swelling was confirmed, but
Carbon dioxide (mmol/liter) 23–32 23
the remainder of the neurologic examination was
normal. A small pink lump was noted on the
Urea nitrogen (mg/dl) 8–25 13
right upper lip, and an ulcer was present on the
Creatinine (mg/dl) 0.60–1.50 0.73 hard palate. There were multiple splinterlike ul-
Glucose (mg/dl) 70–110 66 cerations on the fingers, nonblanching tender
Phosphorus (mg/dl) 2.6–4.5 3.2 patches with a livedoid pattern and excoriations
Calcium (mg/dl) 8.5–10.5 9.6 on the toes, and purple papules on the feet.
Protein (g/dl) Laboratory test results obtained 9 days before
the current presentation are shown in Table 1.
Total 6.0–8.3 7.4
The patient was referred for an ophthalmo-
Albumin 3.3–5.0 4.4
logic examination. Visual acuity without glasses
Globulin 1.9–4.1 3.1 was 20/15 in the right eye and 20/15 in the left
Aspartate aminotransferase (U/liter) 9–32 27 eye. The pupils were equal in size and had a
Alanine aminotransferase (U/liter) 7–33 16 normal response to light. Results of testing with
Alkaline phosphatase (U/liter) 30–100 69 the use of an Amsler grid and an Ishihara color
Total bilirubin (mg/dl) 0–1.0 0.3
plate were within normal limits. Humphrey
visual-field testing revealed enlarged blind spots
Thyrotropin (μIU/ml) 0.40–5.00 1.18
in both eyes. Applanation tonometry revealed an
IgG (mg/dl) 614–1295 873 intraocular pressure of 11 mm Hg in the right
IgA (mg/dl) 69–309 191 eye and 10 mm Hg in the left eye. On external
IgM (mg/dl) 53–334 114 examination, the eyelids and ocular adnexa were
Cholesterol (mg/dl) normal. Results of a slit-lamp biomicroscopic
Total 0–200 215 examination were normal in both eyes. A dilated-
pupil fundus examination revealed extensive bi-
Low-density lipoprotein 40–130 134
lateral optic-disk edema (grade 4 on the Frisén
High-density lipoprotein 35–100 60
scale, which ranges from 0 to 5, with higher
Triglycerides (mg/dl) 0–150 103 grades indicating more severe edema) and associ-
* To convert the values for urea nitrogen to millimoles per liter, multiply by 0.357.
ated subretinal fluid, as well as peripapillary folds
To convert the values for creatinine to micromoles per liter, multiply by 88.4. in the left eye and multiple peripapillary hemor-
To convert the values for glucose to millimoles per liter, multiply by 0.05551. To rhages in both eyes. There were splinter and
convert the values for phosphorus to micromoles per liter, multiply by 0.3229.
To convert the values for calcium to millimoles per liter, multiply by 0.250. To
subretinal hemorrhages and changes in the
convert the values for bilirubin to micromoles per liter, multiply by 17.1. To con‑ macular pigment in both eyes.
vert the values for cholesterol to millimoles per liter, multiply by 0.2586. To A diagnosis was made.
convert the values for triglycerides to millimoles per liter, multiply by 0.01129.
† Reference values are affected by many variables, including the patient population
and the laboratory methods used. The ranges used at Massachusetts General Hos­ Differ en t i a l Di agnosis
pital are for adults who are not pregnant and do not have medical conditions that
could affect the results. They may therefore not be appropriate for all patients.
Dr. Dean M. Cestari: Approaching a complex clini-
cal case is like solving a medical jigsaw puzzle.
her father had valvular heart disease, and her We identify the key pieces of the case and see
maternal grandmother had had a stroke. Her sis- how they relate to one another while applying
ters were healthy, and there was no family history overarching guiding principles that will allow us
of neurologic, ophthalmologic, or rheumatologic to arrive at the correct diagnosis. There are
disease. many details in this case, some of which might
On examination, the temperature was 37°C, lead to a diagnostic error. Therefore, I will con-

284 n engl j med 378;3 nejm.org  January 18, 2018

 Case Records of the Massachuset ts Gener al Hospital
struct a differential diagnosis on the basis of
several important features of this patient’s pre-
sentation: the bilateral optic-disk edema; the
intermittent transient vision loss; the positional
headaches, which were exacerbated with bending
over; the pulsatile tinnitus; and the weight gain
of more than 20 kg.
Optic-Disk Edema
Does this patient have true optic-disk edema?
The 10 signs of true optic-disk edema can be
assessed on a fundus examination.1,2 The 5 me-
chanical signs are loss of the physiologic cup,
elevation of the disk, blurring of the disk mar-
gins, edema of the nerve fiber layer, and folds of
the retina or choroid. The 5 vascular signs are
hyperemia of the disk, venous dilatation and
tortuosity, peripapillary hemorrhages, infarct
of the nerve fiber layer (cotton-wool spots), and
exudates. In this patient, the bilateral optic-disk
edema and the findings of retinal folds and
peripapillary hemorrhages on the fundus exami-
nation suggest the edema is true optic-disk
edema rather than pseudopapilledema, a condi-
tion that simulates some of these features but is
caused by an underlying, often benign congeni-
tal process.
True optic-disk edema can be distinguished as
either papilledema or papillitis. Papilledema is
optic-disk edema that is caused by increased
intracranial pressure, whereas papillitis is optic-
disk edema that is caused by an optic neuropa-
thy or damage to the optic nerve. The character-
istic features of papillitis due to an acquired
optic neuropathy include decreased visual acuity,
loss or impairment of color vision (dyschroma-
topsia), a relative afferent pupillary defect, and
visual-field defects. In affected patients, the optic-
disk swelling is usually unilateral, with the ma-
jor exception being swelling due to hypertensive
crisis. The patients usually have symptomatic
blurry vision and may report other vision symp-
toms. In this patient, the normal blood pressure
is not consistent with a hypertensive crisis, and
the normal visual acuity, color vision, and pupil-
lary responses are not consistent with papillitis
due to an acquired optic neuropathy.
In contrast, patients with papilledema ini-
tially have no vision symptoms and usually have
normal visual acuity, color vision, and pupillary
responses, with no relative afferent pupillary
n engl j med 378;3 nejm.org  January 18, 2018
defect.3 Visual-field testing commonly reveals
enlarged blind spots in both eyes, and as the
disease progresses, the peripheral nasal fields
become affected.4 All these features were seen
on ophthalmologic examination in this patient,
which suggests that her bilateral optic-disk edema
is in fact papilledema. Central visual-field defects
develop late in the course of papilledema, and
these are usually accompanied by decreased vi-
sual acuity and dyschromatopsia. A relative affer-
ent pupillary defect is present only in highly
asymmetric cases, in which the optic-disk edema
and subsequent vision loss are worse in one eye.
In this patient, the normal visual acuity and
color vision and the finding of isolated enlarged
blind spots that spare the peripheral and central
visual fields are consistent with a relatively re-
cent onset of papilledema.
Transient Vision Loss
This patient also had transient vision loss, which
can have an ischemic or a nonischemic cause.
Amaurosis fugax is painless temporary vision
loss that typically occurs in one eye and is usu-
ally caused by carotid or cardiac emboli to the
retinal circulation. Affected patients often de-
scribe a phenomenon resembling a curtain com-
ing down over the visual field that lasts from 30
seconds to a few minutes. This patient reported
episodes of transient vision loss in both eyes
that lasted only 5 to 10 seconds, a finding that
is inconsistent with amaurosis fugax.
Nonischemic causes of transient vision loss
include abnormalities of the ocular surface (e.g.,
dry eye and corneal epithelial disease), vitreous
floaters, and fluctuating blood sugar. None of
these conditions seem to be likely in this pa-
tient. Episodes of painless vision loss that last
only seconds and are aggravated by changes in
position, such as bending over, are referred to
as transient visual obscurations. The brevity of
transient visual obscurations distinguishes them
from amaurosis fugax and other considerations,
such as migraine. Transient visual obscurations
occur in the context of optic-disk edema (either
papilledema or papillitis) and are thought to re-
sult from changes in position that affect perfu-
sion to the swollen optic-nerve heads. They are a
common symptom in patients with papilledema5
and most likely explain the transient vision loss
in this patient.
285
 The n e w e ng l a n d j o u r na l
Positional Headaches with Pulsatile Tinnitus
Headaches caused by increased intracranial pres-
sure can be similar to migraines, which are uni-
lateral throbbing headaches that are associated
with photophobia, phonophobia, nausea, and
vomiting and are often preceded by a visual aura
with a scintillating or zigzag pattern that lasts
for 20 to 60 minutes.6 Unlike migraines, head-
aches caused by increased intracranial pressure
may be accompanied by tinnitus characterized
by a whooshing noise in one or both ears that
occurs in synchronization with the pulse. The
headache and tinnitus are typically worse when
the patient is lying flat and better when the pa-
tient is sitting or standing. Headaches caused by
increased intracranial pressure are often accom-
panied by transient visual obscurations, which
were most likely present in this patient. These
headaches are also occasionally associated with
binocular horizontal diplopia due to a sixth-nerve
palsy. Headaches caused by increased intracra-
nial pressure do not necessarily occur in one
specific area. Some patients with increased intra-
cranial pressure have pain behind the eyes that
may be exacerbated by eye movement, whereas
others report pain that is located at the back of
the head or begins on one side.4,7
This patient’s history of positional headache,
probable transient visual obscurations, and pul-
satile tinnitus is consistent with a diagnosis of
increased intracranial pressure. The eye exami-
nation revealed normal visual acuity and color
vision, with no relative afferent pupillary defect.
The visual fields had enlarged blind spots. There-
fore, the best explanation for this patient’s pre-
sentation is optic-disk edema and transient vi-
sual obscurations caused by papilledema, which
is due to increased intracranial pressure.
Intracranial Hypertension
What is causing the increased intracranial pres-
sure in this patient? Increased intracranial pres-
sure that does not have a defined cause, which
is known as idiopathic intracranial hypertension
or pseudotumor cerebri, is a disorder that pre-
dominantly affects obese women of childbearing
age.8,9 However, increased intracranial pressure
can be secondary to brain tumors, cerebral venous
thrombosis, and connective-tissue disorders asso-
ciated with vasculitis, such as SLE and Behçet’s
disease. Although the ulcers of the mouth and
286 n engl j med 378;3 nejm.org  January 18, 2018
of m e dic i n e
hard palate seen in this patient are not consis-
tent with the aphthous ulcers of Behçet’s dis-
ease, they are consistent with the ulcers of SLE.
Furthermore, the findings on MRI and CSF
analysis that had been obtained 21 months be-
fore the current presentation were suggestive of
an SLE-related vasculitis or, less likely, meningitis.
The skin lesions are consistent with SLE-related
vasculitis, which has been associated with an
increased risk of the development of cerebral
venous thrombosis.
Repeat MRI of the head with gadolinium en-
hancement should be performed to rule out un-
derlying causes of increased intracranial pressure,
and computed tomography (CT) with contrast
enhancement or magnetic resonance venography
should be performed to rule out cerebral venous
thrombosis. If these tests are negative, a lumbar
puncture should be performed for measurement
of the opening pressure and CSF analysis to de-
termine whether there is evidence of aseptic
meningitis due to SLE. If the opening pressure
is increased and the results of CSF analysis are
normal, a diagnosis of idiopathic intracranial
hypertension is possible. If the CSF white-cell
count and protein level are elevated, as they were
during the first CSF analysis, the diagnosis is
most likely related to SLE.10,11 Given this patient’s
suspected history of SLE and previous episode
consistent with aseptic meningitis, I suspect that
she most likely had increased intracranial pres-
sure associated with SLE, which may be exacer-
bated by the use of glucocorticoids.
Dr . De a n M. Ce s ta r i’s Di agnosis
Intracranial hypertension associated with sys-
temic lupus erythematosus, with a possible con-
tribution of glucocorticoid use.
A ddi t iona l Di agnos t ic S t udie s
Dr. Cunnane: On the current presentation, MRI of
the head was performed (Fig. 2). FLAIR images
showed resolution of the hyperintensity that had
been seen 21 months earlier. Diffusion-weighted
images showed hyperintensity of the optic-nerve
heads and prominence of the CSF space sur-
rounding the optic nerves. Both of these find-
ings can be seen in association with papilledema.
CT venography (Fig. 2) revealed a dominant right
 Case Records of the Massachuset ts Gener al Hospital

A B C

Figure 2. Imaging Studies of the Head Obtained on Presentation.

MRI was performed at the time of the current presentation. Diffusion‑weighted images obtained at the level of the
orbits show hyperintensity of the optic‑nerve heads bilaterally (Panel A, arrow) and prominence of the CSF space
surrounding the optic nerves (Panel B, arrow). In addition, CT venography with contrast enhancement was performed
and revealed a dominant right transverse sinus. A posterior view of the reconstruction shows narrowing of the distal
right transverse sinus (Panel C, arrow), with no evidence of dural venous sinus thrombosis.

transverse sinus, with narrowing of the distal
transverse sinuses bilaterally, a finding seen in
idiopathic intracranial hypertension.12 There was
no evidence of dural venous sinus thrombosis.
Dr. Joseph F. Rizzo III: In the absence of struc-
tural intracranial abnormalities, the next diag-
nostic test was a lumbar puncture, which revealed
an opening pressure of 45 cm of water (normal
range, 10 to 25). CSF analysis revealed only one
nucleated cell per cubic millimeter, which was
within normal limits, and a normal protein level.
This patient meets many criteria for the diag-
nosis of idiopathic intracranial hypertension.8
First, she had signs and symptoms of increased
intracranial pressure, including papilledema, an
opening pressure of more than 25 cm of water,
and normal results of CSF analysis. Second,
there were no localizing findings on the neuro-
logic examination (except for those known to be
caused by increased intracranial pressure, typi-
cally due to sixth-nerve palsy but occasionally
due to fourth-nerve or seventh-nerve palsy), and
she had a normal level of consciousness. Finally,
there were no structural deformities that would
cause increased intracranial pressure or alterna-
tive explanations for increased intracranial pres-
sure. Taken together, the features of this patient’s
presentation, the findings on imaging studies,
and the results of CSF analysis are most consis-
tent with a diagnosis of idiopathic intracranial
hypertension.
However, other features of this patient’s pre-
sentation call into question the diagnosis of an
idiopathic condition. First, we know that she
had received a high dose of prednisone, which
was gradually tapered over a long period. Sec-
ond, we know that 21 months before the current
presentation, CSF analysis had revealed 16 nucle-
ated cells per cubic millimeter, which is consis-
tent with a diagnosis of meningitis. This finding
is potentially important because meningeal in-
flammation increases the risk of the develop-
ment of elevated intracranial pressure due to
reduced absorption of spinal fluid. However, on
the most recent examination, this patient had a
high opening pressure and normal results of CSF
analysis, findings that rule out meningitis as an
explanation of her current increase in intracra-
nial pressure. Third, in the past, this patient had
received a presumptive diagnosis of CNS vasculi-
tis, which can be associated with increased in-
tracranial pressure. Despite these factors, I chose
to treat this patient as though she had the idio-
pathic form of the disease, while being mindful
of relevant contributing factors that could poten-
tially alter the treatment at some future time.
Discussion of M a nagemen t
The management of idiopathic intracranial hyper-
tension usually consists of weight-loss strategies
in combination with medications that inhibit
CSF production. Acetazolamide, a carbonic an-
hydrase inhibitor, is by far the most commonly

n engl j med 378;3 nejm.org January 18, 2018 287

 The n e w e ng l a n d j o u r na l
used medication; other choices include topiramate
and, less commonly, furosemide. Acetazolamide
was initiated in this patient, and the transient
visual obscurations resolved. I also encouraged
her to lose weight and offered strategies to help
her do so.
At a follow-up examination 1 month later, the
patient said, “My vision is perfect.” However, pul-
satile tinnitus was present, along with a head-
ache on the right side that was distinct from her
headache at presentation. Papilledema had de-
creased to Frisén grade 3. Because the tinnitus
had worsened, the acetazolamide dose was in-
creased, and nortriptyline was added to mitigate
the risk of the development of migraines. With
these changes in the regimen, the pulsatile tin-
nitus and headaches decreased, but the papill-
edema did not. Calcium phosphate (carbonate
apatite) kidney stones, which are a complication
of acetazolamide use, developed. The patient’s
urologist prescribed potassium citrate to reduce
the risk of subsequent kidney stones.
Nine months later, the patient had lost 14 kg.
An eye examination revealed that the blind spots
had decreased in size and the papilledema had
decreased slightly. Additional kidney stones de-
veloped, leading to surgical removal and a re-
duction in the acetazolamide dose. I also added
topiramate to her regimen to further reduce the
acetazolamide dose. Results of automated visual-
field testing were normal. I also performed opti-
cal coherence tomography of the optic nerve to
obtain volumetric measurements of the optic-
nerve head, which can be helpful in monitoring
patients with idiopathic intracranial hyperten-
sion. Interpretation of the results of optical co-
herence tomography of the optic-nerve head re-
quires nuance, because a decrease in volume can
result from either a decrease in papilledema or a
loss of retinal ganglion-cell axons due to persis-
tent papilledema.
Dr. David M. Dudzinski (Medicine): Dr. Stone, how
did the rheumatology team treat this patient?
Dr. Stone: By the time this patient presented
with idiopathic intracranial hypertension, she had
already begun to receive therapy that would ulti-
mately control her SLE and — perhaps more
important with regard to idiopathic intracra-
nial hypertension — limit glucocorticoid-related
weight gain. Although B-cell depletion therapy
is a highly effective glucocorticoid-sparing treat-
288 n engl j med 378;3 nejm.org  January 18, 2018
of m e dic i n e
ment for some forms of systemic vasculitis, it
has been unsuccessful to date in clinical trials
involving patients with SLE. Nevertheless, many
experts in SLE believe that B-cell depletion is
effective in carefully selected patients with SLE.
The vasculitic nature of SLE in this patient, the
failure of conventional immunosuppressive agents
to control her disease, and the concerns about
the toxicity of continued use of high-dose pred-
nisone and cyclophosphamide led us to try B-cell
depletion therapy. We selected an empirical
remission-induction regimen of two 1-g doses of
rituximab separated by 2 weeks, followed by one
1-g dose every 4 months.
This regimen has permitted us to taper the
prednisone dose, with good control of the cuta-
neous vasculitis and no sign of CNS vasculitis.
After the prednisone dose was tapered, the pa-
tient was able to lose a substantial amount of
weight. These interventions, plus the combina-
tion of acetazolamide and topiramate, have con-
trolled her idiopathic intracranial hypertension.
The relationship between SLE, idiopathic intra-
cranial hypertension, and glucocorticoids is com-
plex. Although idiopathic intracranial hyperten-
sion has been reported in patients with SLE, no
consistent mechanism for this complication has
been defined, and most patients with SLE and
idiopathic intracranial hypertension who have
been described in the literature have also re-
ceived lengthy courses of high-dose glucocorti-
coids.10,11 Therefore, in most cases, it is not clear
which factor — SLE or glucocorticoid use (with
associated weight gain) — is the precipitating
factor. In this patient, I suspect that the reduc-
tion in the glucocorticoid dose, with corre-
sponding weight loss, was the crucial element in
her recovery.
Fina l Di agnosis
Intracranial hypertension associated with system-
ic lupus erythematosus and glucocorticoid use.
This case was presented at Ophthalmology Grand Rounds at
Massachusetts Eye and Ear.
Dr. Stone reports receiving grant support and consulting fees
from Roche–Genentech and Xencor. No other potential conflict
of interest relevant to this article was reported.
Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.
We thank Dr. Suzanne Freitag for assistance with organizing
this conference.
 Case Records of the Massachuset ts Gener al Hospital

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