Background: Disease stratification in GCA is an urgent need, with patients categorised into cranial and large-vessel GCA (LV-GCA) subgroups. LV-GCA may have worse outcomes with regards relapsing disease, poor response to glucocorticoids (GC) and aortic involvement.

Objectives: We report a single centre experience using clinical, imaging and treatment outcomes from a specialist clinic.

Methods: 134 patients with LV-GCA were identified over a 10-year period at Southend University Hospital (2012-2022). Medical records were reviewed retrospectively for baseline demographics, clinical presentation, inflammatory markers, imaging (vascular ultrasound, PET-CT, echocardiography), vascular damage and treatment.

Results: There was a female predominance (female n=91). Age at presentation ranged from 46 to 86 years (median 70 years). Co-morbidities implicated in aortic disease included hypertension (n=60), hypercholestrolaemia (n=29), diabetes (n=14), aortic valvular disease (n=5) and atherosclerotic disease including coronary and carotid artery disease (n=19). Constitutional disturbance was most frequently observed presentation (70%, n=94), and the only feature for 11 patients. This was followed by cranial symptoms (62%, n=83), polymyalgia (53%, n=71), ischaemic symptoms i.e., visual disturbance or tongue/jaw claudication (24%, n=32) and cardiovascular presentations (7%, n=9). The latter included limb claudication, stroke, and aortic aneurysm. Although LV-GCA refers to extra-cranial disease, 12 patients (9%) had isolated cranial and/or ischaemic symptoms at initial presentation.

Inflammatory markers were typically elevated at presentation, C-reactive protein ranged from 1-425mg/L and ESR 1-130mm. Vascular ultrasound was used at diagnosis in 93 patients, with positive temporal artery findings in 50% (n=38) and positive axillary findings in 75% (n=57). PET-CT data was available for 125 patients, of which 113 were positive for LV-GCA. Thoracic aorta FDG-uptake was seen in 77%, with 7 ascending and 1 abdominal aortic aneurysm observed. Transthoracic echocardiogram was available for 46% (n=62). Four (6.5%) patients had a dilated aortic root when indexed to height as per British Society of Echocardiography (BSE) guidelines ¹ (SOV (mm/m) > 21.8mm in males and > 20.7mm in females). Values for our patients were 22.6 and 21.2 mm/m for the female patients and 29.2 and 25.2 mm/m for the male patients. Furthermore, 32 patients showed some extent of diastolic dysfunction as per BSE criteria (52%).

All patients received GC as part of their treatment, 60% (n=82) needing one or more DMARDs and 17% (n=23) Tocilizumab for relapsing disease. DMARDs used included Leflunomide (n=63), Methotrexate (n=18), Mycophenolate mofetil (n=3) and Azathioprine (n=1). One patient received cyclophosphamide.

Conclusion: By combined imaging modalities, 11 patients (8%) had evidence of ascending aortic damage. Grade 1 diastolic dysfunction can be age related, so this may be association rather than causation ^2,3 . Over half of patients had not undergone echocardiogram evaluation, so there may be a hidden burden of disease. Many patients required GC-sparing therapy, showing GC alone are often not enough to halt disease progression, and vascular damage was relatively reduced compared to historical reports. The authors feel GCA services should include standardised protocols for early DMARDs, continuing thorough assessment for LV-GCA and vascular damage, including echocardiography, progressing to cross-sectional imaging if indicated.

REFERENCES:

[1]Harkness A. et al. Normal reference intervals for cardiac dimensions and function for use in echocardiographic practice: a guideline from the British Society of Echocardiography”. Echo Research and Practice 7.1 (2020): G1-G18.

[2]Sanders, D et al. Diastolic dysfunction, cardiovascular aging, and the anesthesiologist. Anesthesiology clinics vol. 27,3 (2009): 497-517.

[3]Jakovljevic and Djordje G. Physical activity and cardiovascular aging: Physiological and molecular insights. Experimental gerontology vol. 109 (2018): 67-74.

Disclosure of Interests: None declared