Respiratory tract symptoms may occur in about 9% of patients with GCA and are the initial manifestation in 4% of patients (13). GCA should be considered in an elderly patient with new onset of cough, hoarseness, or throat pain without obvious cause. In a series of 16 patients with respiratory tract manifestations, cough was noted in 11 patients, while 15 patients had a sore or tender throat, 3 had hoarseness, 1 had a sensation of choking, 4 had a sore tongue, and 2 complained of chest pain (13).
Visual symptoms and signs are common in GCA, and blindness is one of its most feared complications. These symptoms may be the initial manifestation of GCA, and in 20% of patients with visual findings, visual loss is the only or initial symptom (3,14,15). Most patients, however, have had complaints consistent with GCA, including headache and jaw pain, as well as fevers, for several weeks or months prior to the onset of visual loss. Ultimately, permanent partial or complete loss of vision in one or both eyes occurs in 20% of patients, with a range according to series of 10% to 60% (3,14,15). Most patients with GCA-related visual loss have an elevated sedimentation rate and often have thrombocytosis.
Partial loss of vision may be transient or permanent. Patients may describe a sensation of "a shade covering one eye," proceeding to complete blindness. Transient monocular visual loss (amaurosis fugax) precedes permanent visual loss in approximately 44% of patients. This pattern of visual loss resembles that seen in patients with atherosclerotic, cerebrovascular, or heart disease, except that in GCA the visual loss may be transient, bilateral, and postural, and related to a change in body position (3,14,15). Diplopia and visual hallucinations (Charles Bonnet syndrome) rarely may occur. In patients presenting with unilateral symptoms that are left untreated, the other eye frequently is affected within one to two weeks.
The visual loss is caused by ischemia of the optic nerve or optic tracks. It may be due to an arteritis of the ophthalmic or posterior ciliary artery branches and less often is due to occlusion of the retinal arterials. GCA, however, is an unusual cause of retinal artery occlusion, and only about 5% to 10% of patients with this complication have GCA. Branch retinal artery occlusion is less frequent, with vasculitis-related hypoperfusion of the ophthalmic or central retinal arteries. Retinal arterials are not directly affected in GCA. Patients with retinal ischemia have findings of "cotton wool" spots and intraretinal hemorrhages. Postural change, such as bending over or standing up from a sitting position, may result in sludging of blood, detected on funduscopic examination, with return to normal in the supine position.
Anterior ischemic optic neuropathy (AION) is the most common cause of vision loss in GCA (3,14,15). It is usually unilateral but may be bilateral with the second eye involved simultaneously or some days to many weeks following involvement of the first eye. Up to 50% of patients presenting with unilateral blindness will develop blindness in the opposite eye without treatment (3,7,15). The funduscopic findings are of a pale swelling and occasional hyperemia, cotton wool spots, and intraretinal hemorrhages (Fig. 3A and B). Arteritic AION is caused by vasculitic occlusion of the short posterior ciliary arteries to the retrolaminar and laminar portions of the optic disc.
AION due to vasculitis, like GCA must be differentiated from that due to nonarteritic, atherosclerotic disease. Patients with this complication are predominantly female, the sedimentation rate is elevated, and there are constitutional symptoms and other typical symptoms of GCA (16). Improvement is rare, even with high-dose glucocorticos-teroid treatment (3,15,17). In patients with nonarteritic anterior ischemic neuropathy similar in age to those with GCA, the sedimentation rate is normal, and there are no constitutional symptoms (14,15). There is hyperemic disc swelling. Symptoms often occur upon awakening, and in 40% of patients, there may be improvement.
Posterior ischemic optic neuropathy (PION) is a much less common cause of visual loss in patients with GCA than in those with AION (7,14,15). It is frequently confused with retrobulbar optic neuropathy, and visual loss is often not profound. It is caused by disruption of blood flow to the retrolaminar portion of the optic nerve, with infarction of the posterior orbital or intracranial portion of the nerve and vasculitis of the ophthalmic and short posterior ciliary arteries. Both AION and PION can be associated with sectorial choroidal ischemia.
Bitemporal visual loss may also occur when the arteries of the optic chiasm are affected. Occipital lobe infarction from inflammatory disease in the vestibular basilar arteries can lead to bilateral homonymous deficits and cortical blindness. Diplopia is caused by damage to the brain stem. Some of these patients may have strabismus.
FIGURE 3 (A) Ischemic anterior optic neuropathy in a patient with GCA and sudden, nonprogressive visual loss. Left eye with pallid swelling affecting the optic disk. The superior pole is primarily affected with superficial retinal hemorrhages and nerve fiber layer edema. An afferent pupillary defect and an inferior altitudinal visual field defect were present. (B) The unaffected right eye with normal-appearing optic disc. Abbreviation: GCA, giant cell arteritis. Source: Courtesy of James Garrity, MD, Mayo Clinic Rochester, MN, U.S.A.
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