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Background We run a rheumatologist-led, ultrasound-driven giant cell arteritis (GCA) fast track pathway (FTP). Currently, no specific referral criteria are required. Sensitive referral criteria are now needed to safely reduce referrals, which have risen year-on-year (92 in Year 3, 84% greater than Year 1). Methods The following were collected for all patients referred to our GCA-FTP from 1/4/2018-31/3/2019 (Year 3): Final diagnosis of imaging/biopsy confirmed-GCA, unconfirmed clinical-GCA or not-GCA using the electronic patient record to September 2019; Rheumatologist-determined clinical probability of GCA (low, moderate, high) before imaging/biopsy; Presence of ≥ 3 ACR 1990 GCA Classification Criteria (biopsy excluded, adapted to increase sensitivity, see table); Presence of Southend GCAPS≥10 (Based on our clinical experience, polymyalgic symptoms were also counted if patients had recently taken prednisolone for PMR). Results 88 consecutive patients were analysed (total 92: 2 missing data; 2 passed through twice). 25/88 (27%) were diagnosed with GCA 24 were imaging/biopsy confirmed. The single patient with unconfirmed clinical-GCA had high clinical probability, indeterminate ultrasound and biopsy after >1-week glucocorticoid. GCA patients were mean age 77.3y (range 63-94), 76% female, all White, with mean GCAPS 15.3 (range 10-25). Our adaptations of the ACR criteria increased ACR-criteria sensitivity: 24% GCA patients had new generalised headache and 16% had ESR10. 14% referrals were non-White, although GCA is rare in this group. GCAPS was based on a largely White population. If GCAPS did not remove 3 points for alternative diagnoses, then 5 additional non-White patients would have scored ≥10. Conclusion This study externally validates GCAPS≥10 as a screening tool for referrals with possible GCA. It captured all GCA patients and screened out 44% referrals. For this indication, it performed better than a consultant rheumatologist. Despite adaptations to improve sensitivity, ACR criteria missed a quarter of cases. To improve GCAPS specificity when scored by a non-rheumatologist, GCAPS should be adapted to reflect the low likelihood of GCA in non-Whites. We plan to only accept patients with GCAPS≥10, where 3 points are removed for non-Whites if not already removed for alternative diagnoses. We predict referrals will reduce but anticipate referrers will need education to limit over-scoring. Disclosures V. Quick None. M. Hughes None. N. Mothojakan None. D. Fishman None.

A 72-year-old man presented with a short history of headache, jaw claudication, double vision, amaurosis fugax and distended temporal arteries. A diagnosis of giant cell arteritis (GCA) was confirmed on temporal artery ultrasound and temporal artery biopsy. Despite treatment with high-dose oral glucocorticoid (GC) and multiple pulses of intravenous methylprednisolone, his vision deteriorated to hand movements in one eye. 8 mg/kg intravenous tocilizumab, a humanised, recombinant anti-IL-6 receptor antibody, was administered within 48 hours of vision loss and continued monthly, resulting in marked visual improvement within days, as well as sustained remission of GCA. This case suggests a possible role for tocilizumab as a rescue therapy to prevent or recover visual loss in patients with GCA resistant to GC treatment, termed refractory GCA. Further research is required to elucidate the role of intravenous administration of tocilizumab in this setting.

The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton's disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA. Future directions for intervention in the treatment paradigm will be discussed.摘要: 巨细胞动脉炎 (GCA) 的诊疗发生了很大进展。在这篇综述中, 我们通过关键词搜索目前相关的英文文献, 以总结和回顾巨细胞动脉炎的最新进展。所使用的关键词为巨细胞动脉炎; 颞动脉炎; 霍顿病;观察;治疗等。在GCA疾病领域内, 临床医生和患者们最关注的问题是诊断延迟, 诊断不确定性和糖皮质激素 (GC) 的使用情况。好消息是诊疗快速通道的建立, 成像技术以及新治疗的日新月异已经应用于GCA的常规管理。在本文中我们也会探讨治疗、干预策略的新方向。.