British Journal of Rheumatology 1997;36:251–254
CLINICAL FEATURES IN PATIENTS WITH PERMANENT VISUAL LOSS
DUE TO BIOPSY-PROVEN GIANT CELL ARTERITIS
C. FONT, M. C. CID, B. COLL-VINENT, A. LOPEZ-SOTO and J. M. GRAU
Department of Internal Medicine, Hospital Clı́nic i Provincial, University of Barcelona, Barcelona, Spain
SUMMARY
The objective was to determine associated clinical findings in patients with visual loss due to giant cell arteritis (GCA) by means
of a record review of 146 patients with biopsy-proven GCA. Twenty-three (15.75%) patients had lost vision. All of these patients
complained of classical GCA cranial symptoms for an average of 1.3 months, 34.8% had an apparent isolated polymyalgia
rheumatica for an average of 10.8 months and 65.2% had premonitory visual symptoms before visual loss for an average of
8.5 days. A clear delay in diagnosis and treatment was present in 15 patients (65.2%) who complained of at least two classical
cranial symptoms for longer than 3 weeks and/or who had presented premonitory visual symptoms for longer than 72 h before
blindness. Two additional patients lost vision while receiving standard steroid therapy. In conclusion, a high proportion of
patients with permanent visual loss have a delayed diagnosis and treatment. A wider recognition of the disease would potentially
reduce the prevalence of irreversible visual loss among GCA patients.
K : Giant cell arteritis, Horton’s disease, Vasculitis, Visual loss, Blindness, Amaurosis, Amaurosis fugax, Diplopia,
Corticosteroid therapy.
A is the most frequent ischaemic complication
and the main cause of permanent disability among
giant cell arteritis (GCA) patients. It is generally
believed that a prompt institution of corticosteroid
therapy efficiently prevents blindness in this disease.
This statement is based on the observation that since
corticosteroid treatment was introduced, and with a
progressively wider recognition of the disease, the
incidence of permanent visual loss, which was 060%
in early series, has decreased over the years [1].
Moreover, corticosteroid therapy usually reverses
premonitory symptoms such as amaurosis fugax,
diplopia and blurry vision which, untreated, lead to
permanent visual defects in a substantial proportion of
individuals. However, visual impairment still affected
8–22% of patients in recent series [2–4]. This
observation suggests that there is still an underrecognition of the disease or that certain patients are
particularly prone to developing blindness early in the
course of the disease before there is a feasible
opportunity of them being appropriately diagnosed
and treated.
In the present work, we reviewed clinical findings
in patients with permanent visual loss due to
biopsy-proven GCA. Special attention was paid to the
number, nature and duration of classical disease
manifestations at the moment of visual impairment.
The aim of our study was to analyse whether patients
with visual loss had enough typical clinical findings to
allow an earlier diagnosis and treatment.
METHODS
We retrospectively reviewed the clinical records of
146 consecutive patients (102 females and 44 males)
diagnosed at our institution over a 15 yr period
(1980–1995). One hundred and twenty-four (85%)
patients were directly evaluated by at least one of the
authors at the time of diagnosis.
Recorded data included the medical history
with particular emphasis on GCA-related clinical
manifestations, findings at physical examination,
ophthalmic evaluation, treatment received and
outcome.
RESULTS
Characteristics of visual impairment
Twenty-three (15.75%) out of 146 patients evaluated
(16 females and seven males) developed permanent
visual loss. Twelve patients (52.1%) presented unilateral and seven patients (30.4%) bilateral amaurosis.
In two patients from the latter group, blindness was
simultaneous in both eyes. In the remaining five, loss
of vision was sequential and the second eye was
impaired within a 2 week interval. Four patients
(17.4%) presented permanent visual field defects. In 13
patients (15 eyes), visual loss was sudden and in the
remaining nine (14 eyes) progressive blurry vision or
additive visual field defects led to complete blindness in
less than 2 weeks (Table I).
Sixteen patients (65.2%) had premonitory visual
symptoms an average of 8.5 days (range 0.5–60 days)
before permanent visual loss appeared (Fig. 1A). Seven
patients developed blurry vision and/or partial field
defects, six amaurosis fugax, two diplopia and in one
patient there were combinations of these (Table I).
Funduscopy revealed anterior ischaemic optic
neuropathy (AION) in 17 patients (73.9%) which was
bilateral in six of them. In an additional patient, AION
Submitted 3 June 1996; revised version accepted 31 July 1996.
Correspondence to: Maria C. Cid, Department of Internal
Medicine, Hospital Clı́nic i Provincial, Villarroel 170, 08036Barcelona, Spain.
= 1997 British Society for Rheumatology
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BRITISH JOURNAL OF RHEUMATOLOGY VOL. 36 NO. 2
was associated with central retinal artery occlusion.
One patient had an occlusion of a central retinal artery
branch. In another patient, fundus examination was
normal. Funduscopy was not performed in the
remaining three patients.
Systemic symptoms and disease duration before visual
loss
Constitutional symptoms were present in six (26%)
patients, fever in two (8.7%), myalgia in three (13.04%)
and polymyalgia rheumatica (PMR) in 12 (52.1%). At
the time of visual impairment, all patients complained
of cranial symptoms attributable to GCA (headache
in 78.3%, jaw claudication in 56.5%, facial pain
in 30.43%, scalp tenderness in 21.7%, odynophagia
in 17.4%, tongue pain-ischaemia in 13.04%, otalgia
in 8.7%, odontalgia in 4.3% and palate pain in 4.3%
of patients). Twenty patients (86.9%) complained of
more than one cranial symptom (Fig. 1B). The
duration of cranial symptoms before blindness ranged
from 1 week to 4 months (median 1.3 months)
(Fig. 1C). In addition, abnormal findings at physical
examination of the epicranial arteries were present in
19 (86.6%). These included absent or asymmetrical
pulse, tenderness, hardness or nodules. In 18 patients,
these abnormalities involved the temporal arteries and
in one patient the occipital arteries.
Twelve patients had PMR. In four patients
(17.4%), PMR co-existed with cranial symptoms at
disease onset. Eight patients (34.8%) presented with
an apparently isolated polymyalgia and later developed typical cranial symptoms, including blindness. The average duration of PMR before visual loss
was 10.8 months (range 1 month–5 years). None of
these patients were appropriately diagnosed
during this period of time and received only
symptomatic treatment with non-steroidal antiinflammatory drugs.
TABLE I
Visual symptoms in patients with visual impairment due to GCA
Visual loss
N
Monocular
Sudden
12
8
Progressive
Binocular
Sudden
Progressive
Visual fields defect
Sudden
4
7
2
4
3
N, number of patients.
Premonitory
symptoms
(N)
amaurosis fugax
diplopia
blurry vision
inferior hemifield
loss
4
1
3
blurry vision
blurry vision
amaurosis fugax
diplopia
inferior hemifield
loss
1
4
1
1
amaurosis fugax
blurry vision
2
1
2
1
F. 1.—(A) Time with premonitory visual symptoms before
blindness in patients with visual impairment due to GCA. (B)
Number of cranial symptoms in patients with visual impairment due
to GCA. (C) Time with cranial symptoms before visual impairment.
Response to treatment
Eighteen patients were treated with oral prednisone
(1 mg/kg/day) and three patients received high i.v.
doses of methylprednisolone (1 g/day) for 3–5 days. No
patient felt significant improvement in their vision after
treatment. However, two patients with permanent
unilateral visual loss had amaurosis fugax and blurry
vision in the remaining eye which completely reversed
with treatment. The remaining two patients developed
bilateral and unilateral blindness due to AION while
taking 1 mg/kg/day of prednisone, 2 weeks and 5
months, respectively, after starting therapy.
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FONT ET AL.: VISUAL LOSS IN GCA
F. 2.—Proportion of patients in whom the diagnosis of GCA could
have potentially been made before blindness (A), patients in whom
blindness appeared very early in the course of GCA and/or when
only mild cranial symptoms of GCA were present (B), and patients
who presented permanent visual loss in the course of standard steroid
therapy (C).
Delayed diagnosis and treatment
We considered that there had been a clear delay in
diagnosis and treatment when patients had presented
premonitory visual symptoms (amaurosis fugax,
diplopia, sustained blurry vision or partial field defects)
for longer than 72 h and/or at least two classical cranial
symptoms (new-onset headache, jaw claudication,
facial pain or scalp tenderness) for longer than 3 weeks
when visual loss occurred. According to these criteria,
15 (65.2%) patients had a delayed diagnosis and
treatment. Conversely, in six patients (26.09%),
permanent visual defects appeared either very early in
the course of the disease or when only mild
manifestations were present. In addition, as mentioned
above, two (8.7%) additional patients became blind
while receiving corticosteroid therapy (Fig. 2).
DISCUSSION
Even though corticosteroid treatment has clearly
improved the visual prognosis of individuals with
GCA, a substantial number of patients still suffer from
permanent visual defects. In our series of 146 patients,
diagnosed over the last 15 yr, 23 (15.75%) developed
visual impairment, a similar pattern to that reported in
recent reports [2–4]. Once amaurosis is established, the
visual prognosis is known to be poor in patients with
GCA [1, 5–7]. Although several cases of total or partial
visual recovery following corticosteroid treatment have
been reported [3, 5, 8–10], none of our patients
experienced significant visual improvement. Moreover,
two patients lost vision while receiving corticosteroid
therapy.
A careful analysis of associated clinical findings and
disease duration in patients with visual loss disclosed
that in eight patients PMR preceded by an average of
10.8 months the onset of cranial symptoms, including
amaurosis. Similar findings have been reported in other
retrospective series [11]. None of our patients were
previously diagnosed as having PMR, treated accordingly or instructed about the possibility of cranial
symptom development. In prospective follow-up
studies, only a minority of patients considered to have
isolated PMR after a careful clinical evaluation and
appropriately treated with low prednisone doses,
eventually develop full-blown GCA [12, 13]. Therefore,
our patients could theoretically have been diagnosed as
having GCA if an appropriate work-up had been
performed. However, in retrospect, we cannot evaluate
whether at physical evaluation our patients had
abnormalities of temporal arteries or other clinical
findings which could have led to an earlier diagnosis of
GCA during their phase of apparently isolated PMR.
At the time of visual loss, all our patients had cranial
symptoms. Moreover, 16 (65.2%) of our patients had
premonitory symptoms such as amaurosis fugax,
diplopia, blurry vision or partial field defects which
are considered alarming premonitory symptoms of
permanent visual loss. This frequency is similar to
previous reports [1, 14] and higher than reports from
other recent series [2–4]. Fifteen (65.2%) of our
patients had at least two classical cranial symptoms for
longer than 3 weeks and/or classical premonitory
ocular symptoms for longer than 72 h when ischaemic
events occurred. These patients had sufficient clinical
manifestations to allow an earlier diagnosis and
treatment, and visual loss could potentially have been
prevented. In contrast, in six (26.09%) patients, cranial
symptoms had been present for Q2 weeks or consisted
of less classical manifestations, making the possibility
of an early diagnosis and treatment less likely.
In summary, our results indicate that in a high
proportion of patients, an early diagnosis and
treatment could have been carried out, increasing the
likelihood of preventing permanent visual loss, and
suggest that a better awareness of disease manifestations at the primary care level would further reduce the
frequency of blindness in GCA patients. However, in
a remarkable proportion of patients (26.09%), visual
impairment appeared very early in the course of the
disease or when only atypical or mild symptoms were
present without a feasible opportunity of them being
appropriately diagnosed and treated. In addition, two
patients developed bilateral amaurosis during the
course of appropriate steroid therapy. These facts
suggest that some patients are at special risk of
developing ischaemic complications. We are currently
trying to identify prospectively clinical or analytical
data with good predictive value in delimiting patients
at high risk of developing irreversible ischaemic events.
A
Supported by a grant from Fondo de Investigación
Sanitaria (FIS 95/0805).
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