Prior polymyalgia rheumatica is associated with sonographic vasculitic changes in newly diagnosed patients with giant cell arteritis.

OBJECTIVES: To investigate the hypothesis that a history of polymyalgia rheumatica (PMR) is associated with a more severe and damaging disease course in newly diagnosed giant cell arteritis (GCA) patients. METHODS: Retrospective analysis of GCA patients diagnosed between 12/2006 and 05/2021. We compared vascular ultrasound findings (presence of vasculitis and vascular stenosis) in GCA patients with and without prior PMR. RESULTS: 49 of 311 GCA patients (15.8%) had prior PMR in median 30.6 (IQR 7.1-67.3) months before GCA diagnosis. Patients with prior PMR had more often large vessel vasculitis (LVV) (51.0% vs 25.0%, p< 0.001) and stenosis within the vasculitic segments (18.4% vs 3.1%, p< 0.001) on ultrasound. In multivariable analysis, prior PMR remained significantly associated with LVV (OR 7.65, 95% CI 2.72-23.97, p< 0.001). Polymyalgic symptoms at GCA diagnosis in the patients without prior PMR were not associated with a higher prevalence of LVV (p= 0.156). CONCLUSION: Patients with a diagnosis of PMR before GCA diagnosis had two times more often large vessel involvement and significant more vasculitic stenoses on ultrasound examination than patients without prior PMR. Pre-existing PMR is an independent risk factor for more extensive and advanced ultrasound findings at GCA diagnosis. The contribution of subclinical vasculitis to disease associated damage has to be further studied.

Imaging to predict early relapses after treatment discontinuation in patients with large vessel giant cell arteritis - A cohort study.

OBJECTIVES: To investigate the value of [18F]fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) in predicting relapse after treatment discontinuation in patients with large-vessel giant cell arteritis (LV-GCA). METHODS: This study included patients with LV-GCA whose treatment was discontinued between 2018 and 2023. All patients underwent PET/CT and/or MRI at the time of treatment discontinuation in clinical remission. Qualitative and quantitative PET/CT scores, by measuring standardized uptake values (SUV), and semiquantitative MRI scores of the aorta and supraaortic vessels were compared between patients who relapsed within 4 months after treatment discontinuation and those who did not. RESULTS: Forty patients were included (median age 67.4 years, interquartile range (IQR) 60.8-74.0; 77.5 % females). Eleven patients (27.5 %) relapsed after treatment discontinuation (time to relapse 1.9 months, IQR 1.4-3.3). Patients who relapsed were comparable to those who remained in remission with respect to the presence of active vasculitis on MRI and/or PET/CT (54.5% vs. 58.6 %, p = 1.0), the number of segments with vasculitic findings on MRI (0, IQR 0.0-1.5, vs. 2, IQR 0.0-3.0, p = 0.221) or the highest SUV artery/liver ratio on PET/CT (1.5, IQR 1.4-1.6, vs. 1.3, IQR 1.2-1.6, p = 0.505). The median number of vasculitic segments on PET/CT was 2.5 (IQR 0.5-4.5) in those with vs. 0 (IQR 0.0-1.5, p = 0.085) in those without relapse, and the PET/CT scores 4.5 (IQR 0.75-8.25) vs. 0 (IQR 0.0-3.0, p = 0.172). CONCLUSION: PET/CT or MRI at treatment stop did not predict relapse and may not be suited to guide treatment decisions in patients with LV-GCA in remission.

Long delay from symptom onset to first consultation contributes to permanent vision loss in patients with giant cell arteritis: a cohort study.

OBJECTIVES: To characterise factors associated with permanent vision loss (PVL) and potential reasons for the therapeutic delay contributing to PVL in giant cell arteritis (GCA). METHODS: Retrospective analysis of GCA patients diagnosed at the University Hospital Basel between December 2006 and May 2021. RESULTS: Of 282 patients with GCA (64% females), 49 (17.4%) experienced PVL. In 43/49 (87.8%) PVL occurred before treatment. Of these, 24 (55.8%) patients had first non-ocular symptoms and eventually sought consultation when PVL occurred in a median of 21 (IQR 14.75-31.0) days after the first symptoms. Only five of the 24 patients had consulted a physician before PVL, but GCA diagnosis was missed. Treatment was initiated rapidly after diagnosis (median 1 day (IQR 0.0-7.0)). PVL on therapy occurred in six patients in a median of 40 (IQR 20.5-67.3) days after treatment started. In two of those, glucocorticoids were tapered too quickly.In multivariable analysis, patients with PVL were older (OR 1.17, 95% CI 1.07 to 1.29, p=0.001) and reported more frequently jaw claudication (OR 3.52, 95% CI 1.02 to 13.16, p=0.051). PVL was present in 18 (42.9%) of the 42 patients with vasculitic ultrasound findings in all six temporal artery segments. The incidence of PVL over 15 years did not decline (Spearman rank=0.3, p=0.68). CONCLUSION: The prevalence of GCA-associated PVL remains high. Associated factors were advanced age, jaw claudication and ultrasound findings consistent with vasculitis in all six temporal artery segments. Despite preceding non-ocular GCA symptoms weeks before the onset of PVL, most patients were not seen by a rheumatologist before PVL occurred.

Rapid glucocorticoid tapering regimen in patients with giant cell arteritis: a single centre cohort study.

OBJECTIVES: We evaluated the feasibility of a rapid glucocorticoid tapering regimen to reduce glucocorticoid exposure in patients with giant cell arteritis (GCA) treated with glucocorticoids only. METHODS: Newly diagnosed patients with GCA treated with a planned 26-week glucocorticoid tapering regimen at the University Hospital Basel were included. Data on relapses, cumulative steroid doses (CSD) and therapy-related adverse effects were collected from patients' records. RESULTS: Of 47 patients (64% women, median age 72 years), 32 patients (68%) had relapsed. Most relapses were minor (28/32) and 2/3 of those were isolated increased inflammatory markers (19/32). Among major relapses, one resulted in permanent vision loss. The median time until relapse was 99 days (IQR 71-127) and median glucocorticoid dose at relapse was 8 mg (IQR 5-16). Nine of 47 patients stopped glucocorticoids after a median duration of 35 weeks and did not relapse within 1 year. Median CSD at 12 months was 4164 mg which is lower compared with published data. Glucocorticoid-associated adverse effects occurred in 40% of patients, most frequently were new onset or worsening hypertension (19%), diabetes (11%) and severe infections (11%). CONCLUSION: We could demonstrate that 32% of patients remained relapse-free and 19% off glucocorticoids at 1 year after treatment with a rapid glucocorticoid tapering regimen. Most relapses were minor and could be handled with temporarily increased glucocorticoid doses. Consequently, the CSD at 12 months was much lower than reported in published cohorts. Thus, further reducing treatment-associated damage in patients with GCA by decreasing CSD seems to be possible.

A Simplified PET/CT Measurement Routine with Excellent Diagnostic Accuracy for the Diagnosis of Giant Cell Arteritis.

We previously proposed standard uptake value (SUV) ratio-based cut-off values for [18F] fluorodeoxyglucose-positron emission tomography/computed tomography (PET/CT) for diagnosing giant cell arteritis (GCA) with high diagnostic accuracy. Here we confirm our findings in an independent cohort and report a simplified procedure for using a SUV ratio to diagnose LV-GCA. Patients with suspected GCA were consecutively included. The 'peak SUV ratio' was defined in a two-step approach. First, the vessel with the visually brightest radiotracer uptake in the supra-aortic (SA) and in the aorto-iliofemoral (AIF) region was identified. Here, the maximum SUV of the vessel was measured and divided by the mean SUV of the liver (SUVratio). A ratio >1.0 in the SA or >1.3 in the AIF region was scored as vasculitis. The diagnostic accuracy, sensitivity, and specificity of the 'peak SUV ratio' in the SA and AIF region was assessed. From 2015 to 2019, 50 patients (24 female, median age 71 years) with suspicion of GCA were included, 28 patients with GCA and 22 patients with exclusion of GCA. Peak SUV had an AUC of 0.91, a sensitivity of 0.89, and a specificity of 0.73 for diagnosing GCA. Peak SUV accuracy of the AIF arteries was lower (AUC 0.81) than of the SA arteries (AUC 0.95). Our SUV ratio cut-off values for diagnosing GCA are consistently valid, also when applied in a time-efficient clinical procedure focusing on the peak SUV ratio. The diagnostic performance of PET/CT in this validation cohort was even higher, compared to the inception cohort (AUC of 0.83).

18F-FDG PET/CT compared with ultrasound and biopsy for detection of vasculitis of the temporal artery branches.

AIMS: To describe the feasibility and diagnostic accuracy of 18F-FDG positron emission tomography-computed tomography (PET/CT) of the temporal artery compared with temporal artery ultrasound and histology of the temporal artery in patients with suspicion of having giant cell arteritis (GCA). MATERIALS AND METHODS: Patients with suspected GCA were included. PET/CT standard uptake value ratios and the compression sign on ultrasound were assessed for the trunk, and parietal and frontal branches of the temporal artery. Temporal artery biopsies were systematically re-assessed, if available. RESULTS: In 17/34 patients, GCA was confirmed. Temporal artery PET/CT confirmed vasculitis in 9/17 patients and was negative in all 17 controls. Nineteen of 34 subjects had a temporal artery biopsy, which was positive in 7 patients. Five of these seven were negative in the preceding PET/CT. Ultrasound confirmed vasculitis in 9/17 patients and was negative in 16/17 controls. In 7/17 patients, PET/CT and ultrasound were positive for temporal arteritis. Two patients had positive findings only on temporal artery PET/CT and two patients showed vasculitis only on temporal artery ultrasound. No temporal artery segments <1.4 mm were positive on PET/CT. The parietal branches were PET/CT-positive in two patients only. In contrast, on ultrasound vasculitic findings were equally distributed amongst all branches. Sensitivity and specificity for identification of temporal artery involvement was 53% and 100% for PET/CT, and 53% and 94% for ultrasound, respectively. CONCLUSIONS: Assessment of the temporal artery with PET/CT is a valuable extension in the diagnostic workup for GCA. PET/CT and ultrasound have comparable diagnostic accuracy, but differ on a segment and a patient level and may thus be used as complementary tests. PET/CT has a lower sensitivity for the parietal branch than ultrasound and histology.