Visual Manifestations of Giant Cell Arteritis: Trends and Clinical Spectrum in 161 Patients

Abstract

Giant cell (temporal) arteritis (GCA) is the most common systemic vasculitis in Western countries. It involves large and medium-sized vessels with predisposition to the cranial arteries in the elderly. Cranial ischemic complications, in particular permanent visual loss, constitute the most feared aspects of this vasculitis. Although the use of corticosteroids and a higher physician awareness may have contributed to a decrease in the frequency of severe ischemic complications, permanent visual loss is still present in 7%-14% of patients. To investigate further the incidence, trends, and clinical spectrum of visual manifestations in patients with GCA, we examined the features of patients with biopsy-proven GCA diagnosed at the single reference hospital for a defined population in northwestern Spain during an 18-year period. Predictive factors for the development of any visual manifestation, not only permanent visual loss, were also examined. Between 1981 and 1998, 161 patients were diagnosed with biopsy-proven GCA. Visual ischemic complications were observed in 42 (26.1%), and irreversible blindness, mainly due to anterior ischemic optic neuropathy and frequently preceded by amaurosis fugax, was found in 24 (14.9%). Despite a progressive increase in the number of new cases diagnosed, there was not a significant change in the proportion of patients with visual manifestations during the study period (p = 0.37). Patients with visual ischemic complications had lower clinical and laboratory biologic markers of inflammation. Indeed, during the last years of the study, anemia was associated with a very low risk of visual complications. Also, HLA-DRB1*04-positive patients had visual manifestations more commonly. Patients with other ischemic complications developed irreversible blindness more frequently. The best predictors of any visual complication were HLA-DRB1*04 phenotype (odds ratio [OR] 7.47) and the absence of anemia at the time of admission (OR for patients with anemia = 0.07). The best predictors of irreversible blindness (permanent visual loss) were amaurosis fugax (OR 12.63) and cerebrovascular accidents (OR 26.51). The present study supports the claim that ocular ischemic complications are still frequent in biopsy-proven GCA patients from southern Europe. The presence of other ischemic complications constitutes an alarm for the development of irreversible blindness. In contrast, a higher inflammatory response may be a protective factor against the development of cranial ischemic events.

Risk factors for visual loss in an Italian population‐based cohort of patients with giant cell arteritis

“…In our series, the proportion of patients who developed permanent visual loss (19.1%) was similar to that reported by Gonzalez-Gay et al (2,12) and by Cid et al (9) (2,3,9,11). Although geographic and ethnic factors may be implicated in the differences in the incidence of GCA at different latitudes, they do not seem to influence the expression of visual manifestations.…”

“…Furthermore, similarly to our study, an increased risk of developing visual loss was observed in patients with thrombocytosis at diagnosis. Gonzàlez-Gay et al demonstrated an association between the absence of fever and weight loss, on the one hand, and permanent visual loss, on the other; however, acute-phase reactants did not differ significantly between those with visual loss and those without (2,12). These authors also observed a negative association between anemia (Hgb Ͻ12 gm/dl) and the development of severe ischemic manifestations (2).…”

“…Two recent studies from Spain and Israel have reported the presence of irreversible visual loss in 14.9% and 18.3%, respectively, of patients with GCA (2,3). By contrast, less recent studies have reported visual disturbances in 30 -60% of patients (4 -7).…”

Epidemiology of giant cell arteritis and polymyalgia rheumatica

“…The vasculitic involvement of these arteries leads to the typical symptoms and classic clinical features of GCA such as bitemporal headache, jaw claudication, scalp tenderness, or abnormal temporal arteries (tender, nodular, swollen, and thickened arteries with decreased pulses) on physical examination (3). However, ocular ischemic complications, the most feared complications of this vasculitis, are generally early manifestations due to the vasculitic involvement of ocular vessels deriving from the internal carotid artery (4). In unselected patients with biopsy-proven GCA, visual ischemic complications occur in 25% and irreversible visual loss occurs in 10 -15% of the patients (4).…”

“…However, ocular ischemic complications, the most feared complications of this vasculitis, are generally early manifestations due to the vasculitic involvement of ocular vessels deriving from the internal carotid artery (4). In unselected patients with biopsy-proven GCA, visual ischemic complications occur in 25% and irreversible visual loss occurs in 10 -15% of the patients (4). They are generally due to anterior ischemic optic neuropathy that is caused by interruption of blood flow in the posterior ciliary arteries to the optic nerve head.…”