Recommendations for defining giant cell arteritis fast-track clinics

BackgroundThe ultrasonographic (US) Halo Score provide a quantitative measure of the extent of vascular inflammation in patients with giant cell arteritis (GCA). High Halo Scores correlate with systemic markers of...

Background Jaw and tongue pain with constitutional symptoms and raised inflammatory markers are considered pathognomonic ischemic features of giant cell arteritis (GCA). Temporal artery ultrasound (US) (or biopsy) available in GCA fast-track clinics (FTC) for a rapid assessment of patients with suspected GCA. Atypical presentation and negative US or biopsy require further workup to look for an alternative diagnosis. ENT pathology can occur as a mimic of GCA. Herein we discuss two clinical cases of squamous cell cancer tongue presenting with signs and symptoms that resemble GCA. Methods We have put together a case report. Results Case-1: A 58-year-old male with a recent presumed diagnosis of relapsing GCA was referred with worsening visual symptoms and right eye pain despite ongoing steroid treatment (60mg), for consideration of Tocilizumab (TCZ). His initial presentation was 5 months ago with a right parietal and retro-orbital headache and blurred vision. He had a normal eye examination. His C-reactive protein (CRP) was raised (24). He was started on prednisolone 40 mg for GCA with complete resolution of symptoms with normalised CRP within weeks. Several weeks later, symptoms reoccurred. CT brain, abdomen and pelvis was normal and temporal artery biopsy negative. His prednisolone was increased to 60 mg. 6 months later, his jaw and tongue pain worsened, and he was treated with pulsed methylprednisolone. Due to partial response to steroids, he was referred to consider TCZ. He had tender left TMJ with normal temporal artery US. Urgent MRI head and neck revealed a left posterior tongue mass with the histology confirmed poorly differentiated squamous cell carcinoma. He was managed with chemo and radiotherapy. Case-2: A 75 years old female, presented with right scalp pain, tongue pain, painful swallowing and chewing. Her blood investigations were normal except a raised ESR (48) and presumed GCA she was started on steroids (60 mg). she had initially good response but, within a few weeks, her symptoms returned. She was then referred to our FTC. Temporal artery US and biopsy were normal. MRI of the head and neck showed a large mass seen in the right half of the posterior tongue extending into the deep aspect of the anterior tongue. Histology confirmed poorly differentiated Squamous cell carcinoma. She was treated with a combination of chemotherapy and radiotherapy. Conclusion GCA mimics represent a major diagnostic dilemma. FTC helps to stratify the GCA from mimics. Careful evaluation of the history, examination as well as a temporal artery US helps to exclude GCA and aids prompt requesting of appropriate tests to find an alternative diagnosis such as tongue cancers as in our cases. We have now introduced negative weightage for consideration of alternative diagnoses in our GCA probability score. Disclosures A. Kayani None. A. Sebastian None. L. Borukhson None. M. Whitlock None. B. Dasgupta Consultancies; Roche, Sanofi. Grants/research support; Roche.

Background:Giant cell arteritis (GCA) is the most prevalent form of adult vasculitis, with potentially significant risks including blindness. Early detection and prompt initiation of treatment are therefore imperative. According to...

BackgroundThe EULAR recommendations for the use of imaging in large vessel vasculitis establish that an imaging test supported by clinical pre-test probability (PTP) is sufficient for the diagnosis of giant...

BackgroundUltrasound (US) is a valid imaging tool to detect signs of giant cell arteritis (GCA). Although the halo sign has always been considered the most useful finding for GCA diagnosis,...

Vascular ultrasound has been increasingly used to diagnose giant cell arteritis (GCA). The temporal and axillary arteries are commonly evaluated. However, the usefulness of including the subclavian artery remains unclear. This study investigated whether inclusion of the subclavian artery in addition to the temporal and axillary arteries in the ultrasound evaluation of GCA improves the accuracy of the examination beyond ultrasonography of the temporal and axillary arteries alone. We formed a fast-track clinic to use ultrasound to rapidly evaluate patients with suspected GCA. In this cohort study, patients referred for new concern for GCA received a vascular ultrasound for GCA. Subclavian intima-media thickness (IMT) cutoffs of 1.0 and 1.5 mm were retrospectively assessed. Two hundred thirty-seven patients were referred to the fast-track clinic from November 2017 to August 2021. One hundred sixty-eight patients received an ultrasound for concern for new GCA. With a subclavian IMT cutoff of 1.5 mm, inclusion of the subclavian artery did not identify any patients with GCA who were not otherwise found to have positive temporal and/or axillary artery examinations, and at this cutoff, there was 1 false-positive result. A subclavian IMT cutoff of 1.0 mm identified several subjects diagnosed with GCA who had otherwise negative ultrasounds, but most subjects with an isolated subclavian IMT greater than 1.0 mm had false-positive results, and the specificity of this cutoff was poor. Inclusion of the subclavian artery in the ultrasound assessment of GCA at 2 different cutoffs rarely contributed to the accurate diagnosis of GCA and increased the rate of false-positive results.

ObjectiveTo evaluate the impact of cardiovascular risk (CVR) on the diagnostic accuracy of the ultrasonographic (US) Halo Score in patients with suspected giant cell arteritis (GCA).MethodsRetrospective observational study of patients referred to our US fast track clinic with suspected GCA for a 2-year period. The intima-media thickness (IMT) of cranial and extra-cranial arteries and the Halo Score was determined to assess the extent of vascular inflammation. The European Society of Cardiology Guidelines on CV Disease Prevention were used to define different categories of CVR and patients were classified according to the Systemic Coronary Risk Evaluation (SCORE). The gold standard for GCA diagnosis was clinical confirmation after a 6-month follow-up.ResultsOf the 157 patients included, 47 (29.9%) had GCA after a 6-month follow-up. Extra-cranial artery IMT was significantly higher in patients with high/very high CVR than in those with low/moderate CVR, but only among patients without GCA. Non-GCA patients with high/very high CVR had also a significantly higher Halo Score in contrast with low/moderate CVR [9.38 (5.93) vs 6.16 (5.22); p = 0.007]. The area under the ROC curve of the Halo Score to identify GCA was 0.835 (95% CI 0.756–0.914), slightly greater in patients with low/moderate CVR (0.965 [95% CI 0.911–1]) versus patients with high/very high CVR (0.798 [95% CI 0.702–0.895]). A statistically weak positive correlation was found between the Halo Score and the SCORE (r 0.245; c = 0.002).ConclusionsElevated CVR may influence the diagnostic accuracy of the US Halo Score for GCA. Thus, CVR should be taken into consideration in the US screening for GCA.

ObjectivesClinical presentations of giant cell arteritis (GCA) are protean, and it is vital to make a secure diagnosis and exclude mimics for urgent referrals with suspected GCA. The main objective...

AB0382 DOES THE ULTRASOUND IMAGE OF THE LARGE VESSEL WALLS DIFFER IN THE SUBTYPES OF GCA AND PMR OR THERE IS A SYSTEMIC SUBCLINICAL COMMON INFLAMMATION?

One of the unresolved challenges posed in giant cell (temporal) arteritis (GCA) is the detection and monitoring of large-artery complications, particularly aortitis. Recent investigations support vascular magnetic resonance imaging (MRI) studies in this issue. We report our preliminary experience with this imaging technique in the study of the aorta and its proximal branches in patients with GCA and/or polymyalgia rheumatica (PMR). Between 2000 and 2003, six patients with GCA and/or PMR seen in our department were diagnosed with aortitis using vascular MRI studies. In all cases, the study was performed according to a specifically designed protocol that included MRI and MR angiography (MRA). MRI was a hepful non-invasive method for diagnosis of aortitis in all cases, providing accurate information about its extent. In particular, MRI had a higher ability to detect earlier stages of vasculitis disclosing subclinical aortitis in five of the six patients. The main signs of early vascular inflammation observed were vessel wall thickness and oedema (six cases) and increased mural enhancement on postcontrast T1-weighted images (four cases). MRA disclosed lumen changes (stenosis) in two patients. On follow-up studies, whereas vascular stenosis and vessel wall thickness remained invariable, vascular wall oedema and contrast enhancement improved significantly when disease activity decreased. MRI may be a useful technique for diagnosing patients with occult major artery involvement in GCA, whether presenting with classic symptoms of temporal arteritis or PMR. Its utility for monitoring the course of the disease and response to treatment requires further confirmation.

To elucidate epidemiologic and clinical manifestations of Japanese patients with giant cell arteritis (GCA), the first nationwide survey for GCA was conducted in 1998 in Japan. The first questionnaire on GCA for patients treated in 1997 was sent to 10,717 medical departments in Japan. A total of 177 patients were reported. Among the 177 patients, 66 GCA patients with detailed clinical and epidemiologic features on second survey were analyzed. Prevalence in patients 50 years of age and older in 1997 was 1.47 per 100,000 population in Japan. The average age at onset was 71.5 years old. The male:female ratio was 1:1.7. The association with permanent and complete visual loss (6.5%), jaw claudication (15.2%), and polymyalgia rheumatica (PMR) (30.3%) were low in frequency compared with those reported from other countries. All patients were treated with corticosteroids. Only 3 (4.5%) patients were reported as deceased due to other causes. The prevalence of GCA in Japan was revealed to be extremely low compared with other countries. Clinical findings of permanent and complete visual loss, jaw claudication, and PMR were infrequent among Japanese patients with GCA.

AB0439 Contribution of anti-ferritin antibodies to the diagnosis of giant cell arteritis

Molecular analysis of vascular smooth muscle cells from patients with giant cell arteritis: Targeting endothelin-1 receptor to control proliferation.

FRI0200 TIME TO FLARE IN GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA AND REVIEW OF THE LITERATURE

Polymyalgia rheumatica