Clinical Maxillary artery involvement in giant cell arteritis demonstrated by ultrasonography

We describe two cases of giant cell arteritis where involvement of the superficial temporal artery and maxillary artery were demonstrated using colour doppler ultrasonography. Maxillary artery involvement is responsible for the symptoms of jaw claudication and toothache, and even headaches might be due to the involvement of the middle meningeal artery which is a branch of the maxillary artery. The maxillary artery has been difficult to visualise until now. There are international consensus definitions of ultrasonographic abnormalities seen in the superficial temporal artery affected by giant cell arteritis. We have used those definitions to demonstrate hypoechoic changes in the maxillary artery affected by giant cell arteritis. The maxillary artery can be visualised in the infratemporal fossa from an echo window between the condylar and coronoid processes of the mandible. This is the first proof of concept evidence that maxillary arteries can be visualised using bedside ultrasonography in giant cell arteritis

Ultrasonography in the diagnosis and follow-up of giant cell arteritis

Abstract Colour Doppler ultrasonography is the first measure to allow objective bedside assessment of GCA. This article discusses the evidence using the OMERACT filter. Consensus definitions for ultrasonographic changes were agreed upon by a Delphi process, with the ‘halo’ and ‘compression’ signs being characteristic. The halo is sensitive to change, disappearing within 2–4 weeks of starting glucocorticoids. Ultrasonography has moderate convergent validity with temporal artery biopsy in a pooled analysis of 12 studies including 965 participants [κ = 0.44 (95% CI 0.38, 0.50)]. The interobserver and intra-observer reliabilities are good (κ = 0.6 and κ = 0.76–0.78, respectively) in live exercises and excellent when assessing acquired images and videos (κ = 0.83–0.87 and κ = 0.88, respectively). Discriminant validity has been tested against stroke and diabetes mellitus (κ=−0.16 for diabetes). Machine familiarity and adequate examination time improves performance. Ultrasonography in follow-up is not yet adequately defined. Some patients have persistent changes in the larger arteries but these do not necessarily imply treatment failure or predict relapses.</jats:p

P305 Validating diagnostic GCA ultrasonography skills against an expert sonographer

Abstract Background/Aims Ultrasonography has become the recommended first line investigation for suspected giant cell arteritis (GCA). However, it is still not yet widely available in the UK due to a paucity of skilled rheumatologists. Effective training programmes have been developed, but subsequent hands-on practice is needed to become truly proficient. There is no consensus on the logistics of gaining the ‘appropriate’ level of skill. This project demonstrates a potential mechanism for validation against an expert sonographer. Methods Over a 6-month period, 12 scans of patients referred with suspected GCA were sequentially performed by first a trainee (FC) and then an expert sonographer (CM) on the same day. FC had theoretical knowledge and supervised practical experience but had not scanned independently prior to this project. CM has performed &amp;gt;1000 scans. 6 vascular territories considered to be the core GCA US data set (common, frontal and parietal branches of the superficial temporal artery, and the 1st, 2nd and 3rd part of the axillary artery) were marked as either ‘normal’ or ‘halo’. CM was blinded to FC’s results. Inter-observer reliability was calculated for all vascular territories using Cohen’s kappa. Results A total of 144 vascular territories were scanned in 12 patients. Table 1 details the inter-observer variation, with a near perfect level of agreement demonstrated, kappa=0.90 (95% CI 0.83 - 0.98). Conclusion This is the first study that demonstrates a mechanism for validation of ultrasonography skills which will allow GCA US to become a feasible reality in clinical practice. This project demonstrates that after acquiring supervised training, near perfect levels of agreement can be arrived between a trainee and expert. Disclosure F.L. Coath: None. C. Mukhtyar: None. </jats:sec

Maxillary Artery Involvement in Giant Cell Arteritis Demonstrated by Ultrasonography

We describe two cases of giant cell arteritis where involvement of the superficial temporal artery and maxillary artery were demonstrated using colour doppler ultrasonography. Maxillary artery involvement is responsible for the symptoms of jaw claudication and toothache, and even headaches might be due to the involvement of the middle meningeal artery which is a branch of the maxillary artery. The maxillary artery has been difficult to visualise until now. There are international consensus definitions of ultrasonographic abnormalities seen in the superficial temporal artery affected by giant cell arteritis. We have used those definitions to demonstrate hypoechoic changes in the maxillary artery affected by giant cell arteritis. The maxillary artery can be visualised in the infratemporal fossa from an echo window between the condylar and coronoid processes of the mandible. This is the first proof of concept evidence that maxillary arteries can be visualised using bedside ultrasonography in giant cell arteritis. </jats:p

Incidence of primary large vessel vasculitis in Norfolk, UK from 2011 to 2020

Objectives: To report the annual incidence of primary large vessel vasculitis (LVV) in the adult population of Norfolk County, UK, including giant cell arteritis (GCA) (in those ≥50 years) and Takayasu arteritis (TAK). Methods: Individuals diagnosed by histology or imaging who lived in NR1-NR30 postcode districts were included. Validated criteria from 1990 and 2022 were applied for final classification. Population data were available from the Office of National Statistics, UK. Results: 270 individuals were diagnosed with primary LVV over 4.7 million person-years. The annual incidence (95% CI) of primary LVV was 57.5 (50.8, 64.7)/million person-years in the adult population. 227 and 244 individuals were diagnosed with GCA over ~2.5 million person-years using 1990 and 2022 criteria, respectively. The annual incidence (95% CI) of GCA was 91.6 (80.0, 104.3)/million person-years aged ≥50 years using 1990 criteria and 98.4 (86.4, 111.6)/million person-years aged ≥50 years using 2022 criteria. 13 and 2 individuals were diagnosed with TAK over 4.7 million person-years. The annual incidence (95% CI) of TAK was 2.8 (1.5, 4.7)/million person-years using 1990 criteria and 0.4 (0.0, 1.4)/million person-years using 2022 criteria, in the adult population. The incidence of GCA rose sharply in 2017 coincident with the introduction of a fast-track pathway and fell during the pandemic when the pathway was disrupted. Conclusions: This is the first study that reports the incidence of objectively verified primary LVV in the adult population. The incidence of GCA may be affected by the availability of diagnostic pathways. The use of the 2022 classification criteria results in a rise in the classification of GCA and fall in that of TAK

P126 GCA Hospital Standards (GHOST) - making a map of specialised services for the care of giant cell arteritis across England

Abstract Background/Aims The objective of this project is to map services essential to delivering high quality care in giant cell arteritis (GCA) across England, identifying gaps in provision and thereby help to remove inequalities. To do this however, there must first be agreement on what these best practice services and standards are. Methods A steering committee was formed comprising 18 expert representatives from the 13 clinical regions in England, including rheumatology, ophthalmology, allied health professional and patient representation. A modified Delphi process was commenced with each member initially providing five aspects of service they felt were essential for best practice GCA care. From the 90 answers, common themes were identified by creation of a word cloud and then condensed into domains of practice. These domains were then ranked by each member in order of perceived importance. The top 10 domains taken forward for further review were clinical pathways, patient access, Rheumatology involvement, Ophthalmology involvement, ultrasonography provision, temporal artery biopsy provision, PET-CT scan provision, glucocorticoid treatment, patient education and multi-disciplinary team working. Domains identified as separate areas but not quite making it into the top 10 were Tocilizumab provision, audit and governance and research. With the latter two in particular, it was felt these are overarching principles which should run through all aspects of clinical work. Group consultation was undertaken to discuss the relevant aspects, and from this, three quality metrics and one summary statement were devised for each domain. Rheumatology and Ophthalmology provision were amalgamated, as it was felt these were equally as important, with similar requirements. On the first pass of voting all except ‘patient access’ achieved over 75% agreement amongst the steering committee members. After group consultation and amendment, ‘patient access’ also achieved the minimum 75% agreement cut-off. The final statements can be seen in the table below. Results: Conclusion By devising specific quality metrics in addition to the recommendation statements above, it is envisaged these standards can be easily used as an audit tool to identify gaps and development needs in GCA services. Disclosure F.L. Coath: None. M. Bukhari: None. G. Ducker: None. B. Griffiths: None. S. Hamdulay: None. M. Hingorani: None. C. Horsbrugh: None. C. Jones: None. P. Lanyon: None. S. Mackie: None. S. Mollan: None. J. Mooney: None. J. Nair: None. E. O’Sullivan: None. A. Patil: None. J. Robson: None. V. Saravanan: None. M. Whitlock: None. C. Mukhtyar: None. </jats:sec

Quality standards for the care of people with giant cell arteritis in secondary care

Giant cell arteritis (GCA) is the commonest primary systemic vasculitis in adults. It has significant health economic costs and societal burden (1, 2), which is likely to get worse with an aging population. British and European recommendations endorse early specialist review (3, 4). In England, 49% of centres provide a diagnostic ultrasonography service but there is wide variation in access and speed of delivery (5). 34% of hospitals in England did not have any formal clinical pathway for assessing GCA (5). Primary care physicians require pathways (6), and the experience of secondary care physicians suggests that establishing a robust one is difficult (5). Treatment recommendations provide an impetus for improvement in standards of care. Those with auditable metrics provide an even greater driver for change. For example, adoption of national standards for the treatment of early inflammatory arthritis in the United Kingdom has proven to be a significant catalyst for improvement in care (7). We have formed a multidisciplinary group aiming to create standards to bring about similar nationwide improvement in the care of GCA

Relapse after cessation of weekly tocilizumab for giant cell arteritis: a multicentre service evaluation in England

Objectives The National Health Service in England funds 12 months of weekly s.c. tocilizumab (qwTCZ) for patients with relapsing or refractory GCA. During the coronavirus disease 2019 (COVID-19) pandemic, some patients were allowed longer treatment. We sought to describe what happened to patients after cessation of qwTCZ. Methods Multicentre service evaluation of relapse after stopping qwTCZ for GCA. The log-rank test was used to identify significant differences in time to relapse. Results A total of 336 GCA patients were analysed from 40 centres, treated with qwTCZ for a median [interquartile range (IQR)] of 12 (12–17) months. At time of stopping qwTCZ, median (IQR) prednisolone dose was 2 (0–5) mg/day. By 6, 12 and 24 months after stopping qwTCZ, 21.4%, 35.4% and 48.6%, respectively, had relapsed, requiring an increase in prednisolone dose to a median (IQR) of 20 (10–40) mg/day. 33.6% relapsers had a major relapse as defined by EULAR. Time to relapse was shorter in those that had previously also relapsed during qwTCZ treatment (P = 0.0017), in those not in remission at qwTCZ cessation (P = 0.0036) and in those with large vessel involvement on imaging (P = 0.0296). Age ≥65 years, gender, GCA-related sight loss, qwTCZ treatment duration, TCZ taper, prednisolone dosing and conventional synthetic DMARD use were not associated with time to relapse. Conclusion Up to half our patients with GCA relapsed after stopping qwTCZ, often requiring a substantial increase in prednisolone dose. One-third of relapsers had a major relapse. Extended use of TCZ or repeat treatment for relapse should be considered for these patients

Relapse after cessation of weekly tocilizumab for giant cell arteritis: a multicentre service evaluation in England

OBJECTIVES: The National Health Service in England funds 12 months of weekly subcutaneous tocilizumab (qwTCZ) for patients with relapsing or refractory giant cell arteritis (GCA). During the COVID-19 pandemic, some patients were allowed longer treatment. We sought to describe what happened to patients after cessation of qwTCZ. METHODS: Multicentre service evaluation of relapse after stopping qwTCZ for GCA. The log-rank test was used to identify significant differences in time to relapse. RESULTS: 336 GCA patients were analysed from 40 centres, treated with qwTCZ for a median (interquartile range, IQR) of 12 (12-17) months. At time of stopping qwTCZ, median (IQR) prednisolone dose was 2 (0-5) mg/day. By 6, 12 and 24 months after stopping qwTCZ, 21.4%, 35.4% and 48.6% respectively had relapsed, requiring an increase in prednisolone dose to a median (IQR) of 20 (10-40) mg/day. 33.6% of relapsers had a major relapse as defined by EULAR. Time to relapse was shorter in those that had previously also relapsed during qwTCZ treatment (P = 0.0017); in those not in remission at qwTCZ cessation (P = 0.0036); and in those with large vessel involvement on imaging (P = 0.0296). Age ≥65, gender, GCA-related sight loss, qwTCZ treatment duration, TCZ taper, prednisolone dosing, and conventional synthetic DMARD use were not associated with time to relapse. CONCLUSION: Up to half our patients with GCA relapsed after stopping qwTCZ, often requiring a substantial increase in prednisolone dose. One third of relapsers had a major relapse. Extended use of TCZ or repeat treatment for relapse should be considered for these patients.Published version, accepted version (12 month embargo), submitted versionThe article is available via Open Access. Click on the 'Additional link' above to access the full-text