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Objective: We investigated the development of giant cell arteritis (GCA) in patients with prior diagnoses of isolated polymyalgia rheumatica and/or peripheral arthritis (PMR/PA), and the potentially relevant characteristics of both illnesses in such patients., Methods: We retrospectively compared the features of 67 patients at the onset of PMR/PA, and their outcomes, to those of a random group of 65 patients with PMR/PA who did not develop late GCA. We also compared the features and outcomes of patients with late GCA to those of a random sample of patients with more usual GCA (65 with concurrent PMR/PA and 65 without)., Results: Patients with late GCA represented 7.4% of all patients with GCA included in a large hospital-based inception cohort. PMR/PA preceded overt GCA by 27 months on average. Permanent visual loss developed in 10 patients, including 8 of 48 (17%) patients featuring cranial arteritis. A questionable female predominance was the only distinguishing feature of PMR/PA evolving into GCA; late GCA more often featured subclinical aortitis (OR 6.42, 95% CI 2.39-17.23; p < 0.001), headache (OR 0.44, 95% CI 0.19-1.03; p = 0.06), and fever (OR 0.29, 95% CI 0.13-0.64; p = 0.002) less often compared to the more usual form of GCA. Patients with either form of GCA experienced similar outcomes., Conclusion: A cranial arteritis pattern of late GCA is associated with a significant risk for ischemic blindness. However, compared to the usual form of GCA, late GCA is often less typical, with a higher frequency of silent aortitis. Patients with relapsing/refractory PMR may not be at increased risk for late GCA.

Objective: To determine the risk factors for permanent visual loss (PVL) in patients with biopsy-proven giant cell arteritis (GCA) and the usefulness of the factors in clinical practice., Methods: From 1976 through 2015, the clinical charts and laboratory results of 339 patients with biopsy-proven GCA were recorded prospectively at the time of diagnosis. We used multivariable logistic regression analysis to determine which of 24 pretreatment characteristics were associated with PVL., Results: Visual ischemic manifestations occurred in 108 patients, including PVL in 53 (16%), bilaterally in 15 patients (28%). The independent predictors associated with an increased risk of PVL were age (OR 1.06, 95% CI 1.01-1.12, p = 0.01), a history of transient visual ischemic symptoms (OR 2.62, 95% CI 1.29-5.29, p < 0.01), and jaw claudication (OR 2.11, 95% CI 1.09-4.10, p = 0.03). The presence of fever (OR 0.30, 95% CI 0.14-0.64, p < 0.01) and rheumatic symptoms (OR 0.23, 95% CI 0.10-0.57, p = 0.001) were associated with a markedly reduced risk of developing visual loss (3.7% if features were both present). No laboratory variables were independently associated with PVL., Conclusion: The visual ischemic risk of untreated GCA can be readily estimated upon simple clinical findings, but not laboratory variables. However, we did not identify a subgroup of patients carrying no risk of developing visual loss. Glucocorticoid treatment remains, therefore, urgent for any patient with a high clinical suspicion index.