1040-5488/93/7009-0771$03.00/0
OPTOMETRY AND VISION SCIENCE
Copyright © 1993 AMERICAN ACADEMY OF OPTOMETRY

case report

Vol. 70, No. 9, pp. 771-777

Diagnosis and Management of Temporal Arteritis:

A Review and Case Report

THEODORE GROSVENOR,* VICTOR MALINOVSKY,t JOHN GELVIN,$ and

KHASHAYAR TONEKABONI}

School of Optometry, indiana University, Bloomington, Indiana

ABSTRACT

Temporal arteritis is an insidious disease which, if not
recognized and treated with high-dosage oral predni-
sone or intravenous prednisolone, can result in unilat-
eral or even total blindness due to anterior ischemic
optic neuropathy (AION) or closure of the central artery
of the retina. Unfortunately, the symptoms and clinical
signs of temporal arteritis mimic those of a number of
other conditions including angle-closure glaucoma, hy-
pertension, migraine, trigeminal neuralgia, temporo-
mandibular joint syndrome, carotid artery occlusive dis-
ease, Foster-Kennedy syndrome, and _ nonarteritic
AION. When a patient complains of a severe pain in the
temporal region, along with scalp tenderness and a
feeling of malaise or depression—with or without epi-
sodes of transient loss of vision—he or she should be
referred for a diagnostic work-up which includes an
erythrocyte sedimentation rate and a temporal artery
biopsy. We present here a review of the recent litera-
ture concerning temporal arteritis, followed by a report
of an unusual case in which high-dosage prednisone
therapy was effective in relieving the patient’s symp-
toms and lowering the sedimentation rate in spite of a
negative temporal artery biopsy.

Key Words: temporal arteritis, cranial arteritis, giant
cell arteritis, polymyalgia rheumatica

LITERATURE REVIEW

Temporal arteritis, also known as giant cell ar-
teritis and cranial arteritis, has been described by
Henkind and Chambers’ as “the most frequently
misdiagnosed extraocular cause of preventable vis-
ual loss,” having an exceedingly high incidence of
ocular involvement. Most temporal arteritis suffer-
ers are people beyond the age of 55 or 60 years

Received October 28, 1992; revision received April 28, 1993.
*O.D., Ph.D., Member of Faculty, F.A.A.O.

+ 0O.D., Member of Faculty, F.A.A.O.

+0O.D., Member of Faculty.

(with a slight female preponderance) who have a
history of auto-immune disease such as rheumatoid
arthritis. The pathological process is a patchy gran-
ulomatous inflammation in the walls of the large
and medium sized arteries, with fragmentation of
the internal elastic lamina and the presence of giant
cells. The annual incidence of giant cell arteritis
has been estimated by Fledelius and Nissen? as in
excess of 1 per 1000 for people over the age of 50
years; however, they reported that marked visual
loss was found in only 10 of 264 patients diagnosed
as having giant cell arteritis.

Symptoms

Symptoms of temporal arteritis include a severe
headache, usually in the temporal and occipital
regions; scalp tenderness, noted particularly when
combing the hair; a feeling of malaise or depression;
a loss of appetite; claudication of the jaw while
chewing; and possibly a low-grade fever.*""" If the
inflammatory process affects the ophthalmic artery
and its branches, the patient may report one or
more episodes of transient loss of vision.’ In addi-
tion, there may be complaints of pain in the neck,
shoulders, upper arms, and trunk—a condition
known as polymyalgia rheumatica.? Many patients
who have experienced the symptoms of temporal
arteritis fail to volunteer them in a routine history.
Smith‘ has commented that the doctor has to “pull
the symptoms out of the patient.” Keltner® said
that “the most common reason for failure to make
the diagnosis may be the failure to ask the appro-
priate questions.” In a discussion of the systemic
symptoms of giant cell arteritis, Hayreh™ stated:

“I find widespread lack of awareness among phy-
sicians of this important, typical symptom-complex
of giant cell arteritis. It is not at all uncommon in
our experience to find patients who have repeatedly
given a classical history of this disease to their
physicians and the diagnosis was totally missed and
the patient went blind in one or both eyes” (p. 33).

Temporal Arteritis—Grosvenor et al. 771
Ocular Invoivement

The most common ocular involvement in tem-
poral arteritis is anterior ischemic optic neuropa-
thy, which is responsible for the episodes of tran-
sient loss of vision and may even cause permanent
loss of vision. The cause of the anterior ischemic
optic neuropathy (AION) is a loss of blood supply
to the branches of the short posterior ciliary arteries
supplying the circle of Zinn, which surrounds the
optic nerve. It is seen ophthalmoscopically as a
pseudo-papilledema, or pallid papilledema. The
disk appears to be pale, with narrowing of the
retinal arteries and small peripapillary nerve-fiber-
layer hemorrhages (Fig. 1). The optic nerve ische-
mia may be accompanied by an afferent pupillary
defect and may cause an altitudinal field defect,
most commonly in the inferior field.* In some cases
the optic nerve ischemia occurs behind the globe,
causing retrobulbar optic neuropathy, resulting in
anormal appearing disk, but with the characteristic
altitudinal field defect.° If fluorescein angiography
is performed, there may be a delayed choroidal
filling.?? A less common ocular involvement of tem-
poral arteritis is occlusion of the central retinal
artery resulting in complete and usually irreversible
loss of vision in the involved eye. Ocular involve-
ment may be unilateral or bilateral.

Oculo-motor anomalies, which may or may not
be accompanied by diplopia, occur in some cases of
temporal arteritis. In one report,® a patient com-
plained of transient vertical diplopia followed by
horizontal diplopia occurring on extreme rightward
gaze. According to Riech et al.,° ptosis accompa-
nying ophthalmoplegia may suggest temporal arter-
itis, and ophthalmoplegia associated with difficulty
in upward gaze may precede the more classical
symptoms of giant cell arteritis such as headache
or blindness.

Figure 1. Acute pallid disk edema commonly occurring
in temporal arteritic ischemic optic neuropathy. Retinal
nerve fiber layer edema and hemorrhage can also be
present.

772 OPTOMETRY & VISION SCIENCE

Possibility of Blindness in Untreated Cases. In
untreated cases of temporal arteritis, the possibility
of blindness or other severe visual loss is astonish-
ingly high. In a compilation of data taken from 13
studies, Keltner® found that blindness occurred in
36% of untreated cases. In a study comparing out-
comes of patients treated with glucocorticoids to
those treated in the preglucocorticoid era, a 3-fold
increase in bilateral blindness was found for those
patients who did not receive glucocorticoids; fur-
thermore, of those giant cell arteritis patients who
presented with visual symptoms and were not
treated with glucocorticoids, 50% developed bilat-
eral blindness.*

Systemic Manifestations

Systemic manifestations have been reported in a
small percentage of patients diagnosed as having
giant cell arteritis including transient ischemic at-
tacks, cerebral hemorrhage, stroke, aortic arch oc-
clusive disease, ataxia, dementia, and hearing loss.!°
Keltner® has suggested that many cerebral vascular
accidents in the elderly that have been attributed
to arteriosclerosis—as well as peripheral neuropa-
thy of the feet, carpal tunnel syndrome, and sciatic
neuropathy—may be secondary to giant cell arter-
itis. Riech et al.’° stated that cerebrovascular events
are a frequent cause of fatalities in giant cell arter-
itis, but their specific prevalence is unknown. These
authors reported that both the vertebrobasilar ar-
tery system and the carotid artery system have been
found to be responsible for transient ischemic at-
tacks and strokes.

Diagnosis

A tentative diagnosis of temporal arteritis can be
made on the basis of the patient’s symptoms and
ocular signs. The diagnosis is confirmed by the
presence of an elevated erythrocyte sedimentation
rate and by a positive temporal artery biopsy.

Erythrocyte Sedimentation Rate. Of the two meth-
ods used to measure the sedimentation rate—the
Westergren method and the Wintrobe method—
the Westergren method is preferred.* 5 A sedi-
mentation rate below 20 mm/h is usually consid-
ered to be normal. Some controversy exists con-
cerning the sedimentation rate diagnostic of tem-
poral arteritis: a sedimentation rate of 50 mm/h or
greater has been specified by Henkind and
Chambers! and by the American College of Rheu-
matology.’®? However, Smith‘ has stated that a pa-
tient having a sedimentation rate no higher than
80 mm/h may have temporal arteritis; Keltner® has
reported that the sedimentation rate may be normal
early in the course of the illness, but becomes
elevated later in the disease process. Cases of giant
cell arteritis with normal sedimentation rates have
been reported.’® ’” Other possible laboratory find-
ings may be normochromic and normocytic anemia,
and elevation of alkaline phosphatase.

Hayreh" commented that for the past few years,
in addition to erythrocyte sedimentation rate, he
has used C-reactive protein to evaluate and manage
giant cell arteritis patients: a rise in C-reactive
protein level in serum and other body fluids is
produced by inflammatory processes in the body;
combined information on C-reactive protein and
erythrocyte sedimentation rate is very useful in
diagnosis and evaluation of systemic corticosteroid
treatment.

Temporal Artery Biopsy. Even though giant cell
arteritis involves many large and medium sized
arteries throughout the body, the temporal artery
is in an exposed position and is therefore easily
excised. Albert et al.'* have listed four pathological
changes seen in temporal arteritis: (1) occlusion of
the vessel lumen; (2) fragmentation of the internal
elastic lamina; (3) a patchy degeneration and drop-
out of smooth muscle cells in the media; and
(4) the characteristic granulomatous inflammation,
containing various kinds of inflammatory cells in-
cluding giant cells. However, giant cells are not
always seen and are not necessary for the diagno-
sis.

Additional Diagnostic Procedures. Fundus fluo-
rescein angiography and Doppler sonographic ex-
amination have been recommended for the diag-
nosis of temporal arteritis. Mack et al.!? found
delayed choroidal filling (mean filling time of 69 s)
for 18 patients with visual disturbances due to
biopsy-proven giant cell arteritis, 11 of whom also
had AION, as compared to a mean filling time of
5.5 s for 33 patients with nonarteritic AION. Voll-
rath and Gloor’® have recommended that a Doppler
sonographic examination should be performed, to
tule out the possibility of carotid artery stenosis,
before temporal artery biopsy. They made the point
that in the presence of stenosis of the internal
carotid artery, a temporal artery biopsy could result
in sudden death.

Management

Before the availability of glucocorticoids, there
was no effective treatment for temporal arteritis.
These agents mimic the action of cortisol, which is
produced by the human adrenal cortex in response
to the release of the adrenocorticotropic hormone
by the anterior lobe of the pituitary gland. When a
glucocorticoid agent is administered, it inhibits the
release of cortisol by the adrenal cortex and when
it is discontinued, the adrenal cortex must resume
its excretory function. Because cortisol is necessary
for the continuation of life, sudden withdrawal of
glucocorticoid therapy can have disastrous conse-
quences. Glucocorticoid therapy must be initiated
at a sufficiently high dosage to control the inflam-
matory process; once the inflammatory process has
been controlled, the dose must be tapered gradually
so that the adrenal cortex can be reactivated.”

All authorities agree that the treatment for tem-
poral arteritis is the oral or intravenous adminis-
tration of high doses of glucocorticoids. Henkind
and Chambers’ recommend an initial dosage of oral
prednisone in the range of 60 to 120 mg/day, and

suggest that the dosage be increased, if indicated
by a sedimentation rate taken after the first day’s
dosage. Smith* recommends that after an initial
dosage of 50 mg/day, the sedimentation rate should
be checked on a regular basis, and the dosage should
be sufficiently high to keep the sedimentation rate
below 30 mm/h. Hollenhorst (in the Wang and
Henkind report)® stated that he uses about 80 mg
of prednisone per day, in divided doses, for the first
week, and then reduces the dosage to once daily,
aiming at 40 mg/day by the end of the first month
and 20 mg/day by the end of the second month,
and discontinuing treatment by the end of the
fourth month. He stated that as far as he is aware,
none of his patients have lost vision after the fourth
month of the disease. However, other practitioners
find that treatment can be necessary for a period
of several years.‘» 7!

Juchet et al.2° recommend administration of a
bolus of methylprednisolone, with the aim of reduc-
ing the side effects which may otherwise occur.
Their protocol is to use intravenous methylprednis-
olone, 500 mg/day, for 3 consecutive days, followed
by low doses (an average of 22.5 mg/day) of pred-
nisone or an equivalent drug. The method of treat-
ment used by Hayreh" is as follows: for a patient
with no visual symptoms or visual loss, he starts
with at least 80 mg of oral prednisone daily, or even
up to 120 mg, depending on the severity of the
systemic complications, but for a patient who has
had transient loss of vision or more severe visual
loss, he uses megadose prednisolone therapy, begin-
ning with the equivalent of 1 g of prednisone slowly
by intravenous drip, every 6 to 8 h, with 3 to 4 such
doses. This is followed by 120 mg of oral prednisone
daily. Initially, sedimentation rate and C-reactive
protein level are assessed daily, to evaluate the
response of the patient to the therapy. Hayreh
stressed that some patients react very differently
to the therapy than others, stating that determi-
nation of the maintenance dose is a slow, laborious,
and painstaking job over many months.

Adverse Effects of Prednisone Therapy. Temporal
arteritis is one of a small number of conditions in
which the possible adverse effects of high-dosage
prednisone therapy (see Table 1) are outweighed by
the consequences of withholding therapy or of pro-

TaBLe 1. Adverse effects of prednisone therapy.

Effects of short-term therapy
Edema of the hands and face (typical “moon-face” appear-
ance)
Gastrointestinal tract involvement, including peptic ulcer
Muscular weakness, including loss of muscle mass
Immunosuppression (at risk for fungal and other infections)
Increased intraocular pressure (IOP)

Effects of long-term therapy
Sodium and fluid retention; elevated blood pressure
Elevated blood sugar
Osteoporosis
Posterior subcapsular cataracts
Glaucoma

Temporal Arteritis—Grosvenor et al. 773
TABLE 2. Conditions to be ruled out in the differential diagnosis of temporal arteritis.

Condition
Angle-closure glaucoma
Hypertension

Migraine
Trigeminal neuralgia

Temporo-mandibular joint syndrome
Carotid artery occlusive disease

loss of vision
Foster-Kennedy syndrome Papilledema
Nonarteritic anterior ischemic optic neu- Papilledema

ropathy (AION)

viding inadequate dosage.* 1° Smith‘ has stressed
the importance of continuing high-dosage predni-
sone therapy in spite of the presence of side effects
such as black, tarry stools. Fortunately, the more
serious complications of prednisone therapy such
as diabetes, hypertension, and osteoporosis are not
likely to occur with relatively short-term therapy.

Course and Prognosis

According to Raskin and Appenzeller,® temporal
arteritis is a self-limited disease that usually per-
sists for 1 or 2 years, but spontaneous remission
may occur as soon as 6 months, These authors state
that the purpose of the high dosage prednisone
therapy is not to shorten the course of the disease,
but to prevent the occurrence of complications such
as blindness and aortic rupture, by suppressing the
inflammatory arterial component of the disease
until it spontaneously enters its quiescent stage.
Hayreh" warned that the concept of giant cell
arteritis burning itself out in 9 to 12 months is not
at all true: his experience over a period of 15 years
has been that these patients require maintenance
dosage for years. In a review of the records of 98
patients who had had temporal artery biopsy during
a 5-year period, Chmelewski et al.” reported that
mild flare-ups were common after 1 year of therapy,
and that almost one-half of the 98 patients were
still on steroid therapy after 2 or 3 years.

When vision has been lost in one eye, aggressive
prednisone treatment is recommended in order to
prevent a similar loss in the fellow eye. Smith*
points out that even with prednisone treatment,
there is a possibility that the vision in the second
eye will be lost, but the treatment greatly reduces
the chances of this occurring. In a case reported by
Cohen and Damaske,° a patient, already having lost
light perception in her right eye, was treated with
prednisone for several weeks until she discontinued
the treatment on her own volition: 10 days later,
she lost all vision in her left eye, and was thus
totally blind.

Problems in Differential Diagnosis

Some of the symptoms and signs of temporal
arteritis are sufficiently similar to those of other

774 OPTOMETRY & VISION SCIENCE

Similarities in Symptoms or Signs
Unilateral head pain
Early morning headaches

Unilateral headache
Facial pain; “trigger zone”

Pain in temporal area
Transient, uniocular, painless

Additional Diagnostic Features

Gonioscopic evidence of angle closure;
elevated IOP

Elevated blood pressure

Prodromal visual aura; nausea

Pain in area served by maxillary or mandib-
ular branch of trigeminal ganglion

Malocclusion or other mechanical problem

Transient ischemic attacks; cholesterol
plaques in retinal arterioles

Optic atrophy in contralateral eye; due to
frontal lobe tumor or abscess

An absence of the signs and symptoms of
temporal arteritis

disease conditions to bring about a delay in making
the diagnosis, and therefore in initiating the pred-
nisone treatment. Many cases have been reported
in the literature in which irreversible vision loss
has occurred due to delays caused by inappropriate
diagnostic and treatment procedures.*”” !

Conditions having one or more of the same symp-
toms or signs as temporal arteritis are shown in
Table 2. Angle-closure glaucoma is obviously of
particular interest to primary eye care practition-
ers: in our case report, involving a patient with
narrow anterior chamber angles, a diagnosis of an-
gle closure was initially considered but was ruled
out. Hypertension is also of importance, because
patients in the age-range at risk for temporal arter-
itis are also likely to have hypertension. Although
migraine and cluster headache are usually consid-
ered to affect a relatively young age group, Solomon
et al.!> have reported a high prevalence of these
conditions among headache patients over the age
of 65 years and it has been reported that some
patients with biopsy-proven temporal arteritis pre-
sented with occipital (rather than temporal) head
pain.”" Because of the presence of a “trigger zone,”
the facial pain of trigeminal neuralgia may be con-
fused with that of temporal arteritis. Temporo-
mandibular joint syndrome may be confused with
temporal arteritis inasmuch as both conditions in-
volve a claudication of the jaw while eating. Carotid
artery occlusive disease, although not causing a
headache, like temporal arteritis is a major cause
of transient loss of vision.! The appearance of the
optic nerve head in AION, sometimes referred to
as pallid papilledema, can be responsible for a
pseudo-Foster-Kennedy syndrome which, accord-
ing to Miller et al.,” is much more common than
the true Foster-Kennedy syndrome.

When AION is present, it is imperative that it
be diagnosed correctly as either the arteritic or the
nonarteritic form. Hayreh"™ has stressed the fact
that giant cell arteritis is an ocular emergency, and
has listed the following criteria which indicate that
AION, in a given case, is the arteritic rather than
the nonarteritic form: systemic and visual symp-
toms of giant cell arteritis; elevated erythrocyte
sedimentation rate and C-reactive protein level;
early massive visual loss; chalky-white disk swelling
(occurring exceedingly rarely in the nonarteritic
form); AION associated with cilio-retinal artery
occlusion; and massive choroidal nonfilling on fun-
dus fluorescein angiography.

Because the consequences of untreated temporal
arteritis are so severe, eye care practitioners should
suspect its presence whenever an older patient com-
plains of temporal headaches, scalp tenderness, a
feeling of malaise or depression, and particularly if
the symptoms include transient visual disturbance
with amaurosis fugax, diplopia, ptosis, or blurred
vision. Valuable time should not be lost in pursuing
incorrect diagnoses. Prompt treatment with high
dosage of prednisone is imperative, if catastrophic
vision loss is to be avoided.

CASE REPORT

Our patient, a 65-year-old woman, was examined
at the Indiana University School of Optometry’s
Atwater Eye Care Center on October 17, 1991. She
complained of blurred vision at near, and headaches
which were relieved by pressing on her right eye.
She reported a history of high intraocular pressure
(IOP), hypertension, and osteoarthritis. She also
had a history (although not reported at the initial
visit) of shooting pains in the neck, shoulders, upper
arms, and hips (polymyalgia rheumatica) which had
been treated by chiropractic therapy for the past 7
months.

Her refractive error was found to be: right eye
+3.75 D, resulting in 6/6— (20/20—) visual acuity,
and left eye +3.25 D, resulting in 6/6 (20/20) acuity.
The slight reduction in acuity for the right eye was
due to lens vacuoles. All pupillary reflexes were
found to be present and normal: there was no
indication of an afferent pupillary defect. Using the
Van Herrick angle grading method, both eyes were
found to have grade 1 anterior chamber angles.
IOP’s, measured by applanation tonometry, were
right eye 28 mm Hg and left eye 25 mm Hg before
dilation and right eye 29 mm Hg and left eye 28
mm Hg 1h postdilation. Cup/disk ratios were right
eye 0.35/0.45, left eye 0.3/0.3. A small superior-
nasal notch of the right disk was found upon ster-
eoscopic fundus examination. Gonioscopy showed
unoccludable angles with the trabecular meshwork
visible in three of the four quadrants for each eye,
no peripheral anterior synechiae were found, and
the scleral spur was seen in the remaining quadrant.

The patient was rescheduled for follow-up visits
for Humphrey full-threshold fields and measure-
ment of diurnal IOP variation. Full-threshold fields
were found to be normal. Diurnal IOP variation
was found to be within normal limits, varying no
more than 1 or 2 mm Hg for findings taken at 8:30
a.m., 12:00 noon, and 4:30 p.m.

On December 3, 1991—6 weeks after the initial
examination—the patient came to the Eye Care
Center and was seen as a walk-in patient. She
reported that she had awakened that morning with
a severe, persistent headache in the right temporal

area, and she thought she was having a glaucoma
attack. In addition to the temporal headache, she
complained that she was sensitive to light, that her
right eye and face felt numb, and that she had
recently had three similar but milder attacks. Cor-
rected acuities at that visit, and at all subsequent
visits, were right eye 6/6— (20/20) and left eye 6/
6 (20/20). IOP’s were found to be right eye 24 mm
Hg, left eye 19 mm Hg (in the same range as her
previous IOP findings). Because her IOP’s were not
elevated and gonioscopic findings were negative, in
spite of the severe temporal headaches, a diagnosis
of angle-closure glaucoma was ruled out and a ten-
tative diagnosis of temporal arteritis was made. An
erythrocyte sedimentation rate was ordered, and an
appointment was made for the patient to see her
primary care physician. The sedimentation rate,
measured the following day, was found to be 34
mm/h. Against the wishes of the optometric staff,
the primary care physician was unwilling to make
an emergency appointment for the patient, believ-
ing that it was not warranted by the symptoms and
the sedimentation rate. The patient later reported
that during the 6-day period before her appoint-
ment with her physician, the temporal headaches,
light sensitivity, and feeling of numbness persisted.
In addition, she experienced what seemed to be
dizzy spells, in which her vision would swim out of
focus several times each day. Her vision did not
completely black out during these out-of-focus pe-
riods, which lasted less than 1 s.

When the patient was examined by her physician,
on December 9, 1991, the sedimentation rate was
tested again and was 58 mm/h. The physician im-
mediately prescribed oral prednisone, 40 mg (two
20 mg tablets)/day, and arranged for a temporal
artery biopsy, which unfortunately could not be
performed until 10 days later. After taking the 40
mg of prednisone for 2 days, the patient found that
this dosage failed to completely control her tem-
poral headaches, light-sensitivity, and facial numb-
ness; she continued to have the transient episodes
of blurred vision. She telephoned her physician,
who instructed her to increase the prednisone dos-
age to 60 mg (3 tablets)/day. The 60 mg dosage
controlled the temporal headaches and the tran-
sient losses of vision more effectively, and she no
longer was aware of the neck, shoulder, and arm
pains. However, after 3 more days, because of the
persistence of mild headaches, light sensitivity, and
numbness, the dosage was increased to 80 mg/day.
This dosage controlled all of her symptoms and was
continued for the next 12 days, at which time she
began to experience adverse effects of prednisone
therapy including indigestion, soreness of the
mouth, nausea, swelling of the hands and face, and
weakness in the arms and legs. The dosage was
therefore decreased to 60 mg/day, and after an
additional 7 days it was reduced to 40 mg/day.

The temporal artery biopsy was performed, as
scheduled, 10 days after the prednisone therapy was
begun. The surgeon reported that the right tem-

Temporal Arteritis—Grosvenor et al. 775
poral artery, upon palpation, was constricted and
in spasm. However, the pathologist’s report was
negative for the presence of giant cells in sections
of a branch of the temporal artery—the normal
procedure is for the surgeon to excise a portion of
a branch of the temporal artery rather than excising
the temporal artery itself. Doppler sonographic
studies were not considered necessary in this case,
inasmuch as there was no reason to suspect carotid
artery stenosis; fluorescein angiography was not
considered necessary because there were no
ophthalmoscopic signs of anterior ischemic optic
neuropathy.

Because of the negative temporal artery biopsy,
the primary care physician instructed the patient
to begin tapering the prednisone dosage. At this
point she asked to be referred to an internist, whom
she first visited on January 9, 1992. In spite of the
negative biopsy, which tended to make the diagno-
sis of temporal arteritis equivocal, the internist
instructed the patient to continue the prednisone
therapy. At that visit the sedimentation rate was
found to be 12 mm/h. Because of the gastrointes-
tinal effects of prednisone, the internist prescribed
nizatidine (Axid), a histamine H2 blocker, to be
taken with the prednisone. On January 28, 1992
the sedimentation rate was found to be 18 mm/h,
and the internist instructed the patient to decrease
her prednisone dosage to 30 mg/day for the follow-
ing 8 weeks, and then to reduce it to 20 mg/day.

The patient’s ocular health was reassessed after
she had been on prednisone therapy for 6 months,
at which time her pressures were found to be very
little higher than before the prednisone therapy
was begun: right eye 32 mm Hg and left eye 28 mm
Hg. Results of fundus examination and Humphrey
visual field examination were negative.

The prednisone dosage was tapered over a period
of 11 months, during which none of the symptoms
of temporal arteritis returned, with one exception:
when the dosage was decreased to 15 mg/day there
was a recurrence of temporal headaches and the
sedimentation rate increased to 32 mm/h. At this
point, the dosage was increased to 30 mg/day for a
period of 2 weeks, after which the tapering was
resumed, Adverse effects of the prednisone, such as
swelling of the face, gastrointestinal disturbances,
and muscular weakness gradually dissipated during
the 11-month period.

Approximately 4 months after the discontinua-
tion of the prednisone therapy, the patient com-
plained of almost debilitating pains in the shoul-
ders, hips, and trunk, with occasional mild (but
persistent) head pains. Until the head pains oc-
curred, she thought the pains were manifestations
of prednisone withdrawal, but a complete blood
work-up revealed an erythrocyte sedimentation rate
of 69 mm/h. On the basis of her symptoms and the
high sedimentation rate, her medical practitioner
concluded that the polymyalgia rheumatica and
temporal arteritis had returned, and immediately
resumed prednisone therapy beginning with 60 mg/

776 OPTOMETRY & VISION SCIENCE

day. The long-term prognosis, as this paper is sub-
mitted for publication, is for protracted prednisone
therapy but, hopefully, at a relatively low dosage.

Comment. When this patient originally visited
the Eye Care Center, she was concerned that her
blurred vision, headaches, and feeling of pressure
in her eyes could have been due to glaucoma: no
symptoms or signs of temporal arteritis were evi-
dent at that time. However, when she came to the
clinic 6 weeks later with severe temporal and ocular
pain, but her IOP’s were found to be within normal
limits (for this patient), the tentative diagnosis of
temporal arteritis was made. The tentative diag-
nosis was supported by the second sedimentation
rate, performed 6 days after the tentative diagnosis
was made. The episodes of transient loss of vision
began to occur several days before the patient’s
primary care physician was able to see her, so it
is fortunate that prednisone therapy was begun
without waiting for the temporal artery biopsy to
be performed: a delay of another week or more
could have resulted in severe and permanent loss of
vision.

It is worthy of emphasis that in spite of the
apparently normal temporal artery biopsy (per-
formed 10 days after the prednisone therapy was
begun), the internist wisely elected to continue the
prednisone therapy. The importance of treating the
condition as if it were temporal arteritis in the
absence of definite proof to the contrary was dem-
onstrated by: (1) the fact that the prednisone dosage
of 60 mg/day controlled the transient losses of
vision, temporal headaches, and pains in the prox-
imal extremities, and (2) the fact that these symp-
toms returned 3 months later when the dosage was
lowered to 15 mg/day.

Finally, this patient demonstrates the impor-
tance of closely monitoring the prednisone therapy,
on the basis of sedimentation rate and the patient’s
symptoms. At various times during the manage-
ment of the disease, a high sedimentation rate
together with temporal headaches and transient
losses of vision gave evidence that the dosage was
inadequate, whereas a low sedimentation rate to-
gether with pronounced adverse effects indicated
that the dosage could be reduced.

CONCLUSIONS

Because patients often associate headaches with
eyestrain, and because the pain of temporal arteritis
is often in the vicinity of the eye, many patients
will seek out an eye care practitioner in the rela-
tively early stages of the disease process, before
vision loss has occurred. When episodes of transient
loss of vision begin to take place, the eye care
practitioner is likely to be consulted. For these
reasons, optometrists should be aware of the symp-
toms and signs of temporal arteritis and should
waste no time in referring the patient to a neurol-
ogist or internist for a clinical diagnosis and appro-
priate treatment.
ACKNOWLEDGMENT

Dr. Sally Hegeman very kindly assisted the authors in many ways
concerning the pharmacological aspects of the manuscript. Her
assistance was invaluable.

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AUTHOR’S ADDRESS:
Theodore Grosvenor
School of Optometry

Indiana University
800 East Atwater
Bloomington, Indiana 47405

Temporal Arteritis—Grosvenor etal. 777