Temporal
arteritis or giant cell arteritis is a systemic disorder of unknown cause
characterized by an inflammatory obliterative arteritis particularly, but not
exclusively involving branches of the external carotid and ophthalmic arteries.
It is well known as a cause of anterior ischemic optic neuropathy and less well
recognized as a cause of ophthalmoplegia. The most common initial symptom is
headache, often accompanied by diffuse aches and pains (polymyalgia rheumatica).211
Other common symptoms include jaw claudication, fever, anemia, and weight loss.
In the study of Vilaseca et al.,212 the simultaneous presence of
recent onset headache, jaw claudication and abnormalities of the temporal
arteries on physical examination had a specificity of 94.8% compared to the
histological diagnosis and 100% with respect to the final diagnosis.
Solomon
and Cappa213 point out that the headache of temporal arteritis may
clearly involve more than the temporal area and include pain in the temporal,
frontal, vertex and occipital regions. The headache is characterized by gradual
onset, progressing to a diffuse, often severe, aching. The headache may be
intermittent, but usually becomes a prominent, if not daily, feature of patients
with the disorder. The headache usually is constant and perceived as
superficial in the scalp. There may be exquisite tenderness of the scalp and
blood vessels particularly in the temporal region. The headache is usually
worse at night and may be especially aggravated by exposure to cold.14
Sixty‑five
percent of patients are women with the average age at onset of 70 (range 50‑85).
It is a common disorder and must be actively sought in any headache patient
presenting after the age of 50 particularly in those with systemic symptoms.
Allen and Studenski214 emphasize additional symptoms such as
extremity and tongue claudication, ear pain, stroke, and angina as well as
systemic panarteritis involving the peripheral nervous system and abdominal or
pelvic viscera.
Other ocular
complaints with temporal arteritis include tonic pupils from ischemia of the
ciliary ganglia215 and bilateral uveitic glaucoma which may be on an
immunologic basis.135 While most attention has been drawn to
vascular complications in the distribution of the external carotid and
ophthalmic arteries, rarely patients present with vertebral basilar
symptomatology.216 Among the protean manifestations of temporal
arteritis are those described in the following reports: bilateral carotid artery
occlusion,217 renal disease,218 aortic arch arteritis,219
temporal mandibular joint pain,220 painful facial swelling,221
jaw claudication,222 and sudden death from arteritis in the coronary
arteries, dissection of the aorta and major cerebrovascular accident.223
An elevated
sedimentation rate has been considered by some indispensable in diagnosing
temporal arteritis. Without an elevated sedimentation rate, even in patients
with a classic history and clinical findings, the diagnosis might be abandoned
without proceeding to a temporal artery biopsy. Nonetheless, it is now estimated
that up to 9% of patients with temporal arteritis may have normal sedimentation
rates.224,225 Wong and Korn224 have identified 37
reported cases of biopsy‑proven temporal arteritis with a normal Westergren
sedimentation rate (< 40 mm/hr in patients > than 50 years old).
Up to one third of
temporal artery biopsies may be falsely negative, especially when one fails to
examine a long segment of vessel by serial sections. As recently stated,
"because of the danger to vision, most authorities would start steroid treatment
when they suspect the diagnosis clinically. Any response other than prompt and
striking improvement in clinical well‑being and symptoms, however, speaks
against the diagnosis."226 Raskin14and Allen and
Studenski,214 and others believe steroid therapy should start
immediately, before confirmation by laboratory and pathologic determinations.
They suggest in very ill patients, intravenous methylprednisolone may be better