Molly,
72, had a headache like none she had ever experienced. “It
was so bad that my head seemed to throb when I brushed my
hair or put on my reading glasses,” she said. Her
vision seemed increasingly blurry, and one day she suddenly
lost vision completely in one eye for several hours.
The above are symptoms of several very serious illnesses,
all requiring urgent attention. And Molly’s prompt
trip to the doctor may well have saved her sight.
When the doctor said he suspected “temporal arteritis,”
Molly misunderstood. “I didn’t know you could
have arthritis in your eyes,” she said.
As the doctor explained, arthritis is by definition an inflammation
of a joint (arthros–joint plus itis–inflammation).
Arteritis is rather an inflammation of an artery or arteries–in
this case, including those in the temple–above and
in front of the ears.
Temporal arteritis, also known as giant cell arteritis,
is an inflammatory disease within the large and medium-sized
blood vessels. The precise cause is not known, but scientists
believe that it may involve a genetically programmed immune
reaction to an infection. The disease can cause either narrowing
or swelling of blood vessels. In either case, the result
is reduced blood flow.
In addition to severe headaches and scalp tenderness, symptoms
include soreness in the face or jaw, particularly when chewing,
and sometimes fever, weight loss, night sweats, depression,
tiredness and a general feeling of being sick. In addition
to being tender and sore, the arteries in the temple sometimes
appear swollen and pulsating.
Blurred vision, double vision, blindness or a stroke can
occur as a result of decreased blood flow through the arteries
serving the eyes.
When visual problems are treated early, as they were in
Molly’s case, they usually resolve. At least half
of patients who are untreated or treated late end up with
permanent blindness, caused by oxygen deprivation to the
optic nerve and retina.
It’s
Also Arthritis
About half of persons with temporal arteritis, including
Molly, also have aching joints and muscles–a condition
known as polymyalgia rheumatica.
Except for the visual symptoms, the two diseases are similar,
and some believe they may be different manifestations of
the same disease process. In that sense, temporal arteritis
may be considered a form of arthritis after all. Treatment
may be by a rheumatologist (specialist in arthritic diseases),
an ophthalmologist, an internist or a general practitioner.
Symptoms of polymyalgia rheumatica, which may develop fairly
quickly, include aches, pains and stiffness in joints and
large muscle groups, particularly those of the hips, neck
and shoulders. The stiffness is usually worse in the early
morning or after a period of sitting.
Some patients report having to roll out of bed and having
difficulty getting dressed, grooming their hair and brushing
their teeth. As with temporal arteritis, the pain is associated
with poor blood flow to the affected joints and muscles.
Similar immunological responses to those of temporal arteritis
have been found, and scientists believe it may represent
a milder but more widespread reaction to an infection or
other stimulus.
As with arteritis, polymyalgia rheumatica typically brings
with it a low-grade fever, weakness, fatigue and weight
loss.
Diagnosis of either illness involves a physical examination
plus blood tests. A good indication for either illness is
an elevated ESR (erythrocyte sedimentation rate).
ESR measures the time it takes for red blood cells to collect
as sediment in the bottom of a test tube. An abnormally
rapid rate is an indication of inflammation.
Normal ESR is about half a patient’s age (slightly
higher for a female). A patient with temporal arteritis
may have a reading of 80 to 100 millimeters/hour or higher.
CRP (c-reactive protein), a protein produced by the liver
in response to injury, is another marker for systemic inflammation,
and CRP is usually high as well in patients with either
disease, particularly temporal arteritis.
A biopsy of the temporal artery (cutting out a small segment
to be examined under the microscope) may be used to confirm
arteritis. A negative biopsy, however, does not necessarily
rule out the disease since the inflammation may have a rather
splotchy distribution through the blood vessel.
With treatment, either disease will usually resolve within
two to three years. While polymyalgia rheumatica may cause
more short-term suffering, temporal arteritis is the more
dangerous of the two diseases. Without effective treatment,
it can lead to permanent loss of sight plus an increased
risk of a stroke or a life-threatening aortic aneurysm.
Corticosteroids such as prednisone are used to treat either
illness, but the difference in approach is critical. Patients
with polymyalgia rheumatica alone are usually given nonsteroidal
antiinflammatory medications first. If these don’t
work, a low dose of corticosteroids is usually effective.
Temporal arteritis requires high doses of corticosteroids
started immediately to suppress the inflammation and reduce
the risk of blindness. In most cases, hospitalization is
not required.
Although symptoms may clear rather quickly, doctors sometimes
recommend continuing the high dose until the ESR comes back
to normal–usually four to six weeks. After that time,
the dose can be tapered gradually so that the adverse effects
of high-dose corticosteroid therapy can be avoided. Careful
monitoring for signs of a relapse is needed during this
tapering off period. A low dose may then be continued for
18 to 24 months.
Corticosteroids can have severe side effects so it’s
important to limit the dose as much as possible. But it’s
even more critical to resolve the arteritis as quickly as
possible to remove the threat to vision.
In recent years, methotrexate has been used in treatment,
either in conjunction with prednisone or as an alternative.
In the search for new therapies, researchers are looking
at the role of the immune system and the genetic and/or
environmental factors that link the two diseases.
For both of these inflammatory diseases, the average age
of diagnosis is 70. Although not household terms, they’re
both relatively common, with temporal arteritis affecting
as many as 1 in 500 persons age 50 and over and polymyalgia,
1 in 200.
As Molly learned, “arthritis of the eyes” is
not to be taken for granted as a natural consequence of
aging but an urgent medical problem. With decisive treatment,
the danger can be removed.
