Giant Cell Arteritis Classification Criteria Research Articles

Characteristics associated with anterior ischemic optic neuropathy (AION) in giant cell arteritis (GCA).

Giant cell arteritis (GCA) is the most common vasculitis and can result in blindness due to anterior ischemic optic neuropathy (AION). Little is known about which patients with GCA are at higher risk of AION.We did a retrospective chart review to compare demographics, past medical history, labs and imaging of GCA patients with and without AION. All patients at the University of Washington who were diagnosed with GCA by a rheumatologist, had a vascular ultrasound and met classification criteria for GCA were included. AION was diagnosed by an ophthalmologist. To compare demographics, symptoms, presentation imaging and lab findings, we used Pearson Chi-square, Fisher's exact test and t-tests to compare GCA patients with and without AION. 91 patients with GCA without vision loss were compared to 15 patients with GCA and AION.The AION group had significantly more men (p=0.03) and elevated C-reactive protein (CRP) as compared to the non-AION group (p = 0.04). Rates of hypertension, smoking, erythrocyte sedimentation rate (ESR) and classification scores were similar. Vascular ultrasound showed similar rates of small and large vessel vasculitis, but the AION group had higher halo scores, with the AION group having a mean score of 5 and patients with GCA and without AION having a mean score of 3 (p < 0.01). Older men with GCA and more than three halos on vascular ultrasound imaging of GC may be at higher risk of AION. Key Points • This is the first paper to incorporate the use of vascular ultrasound in comparing patients with anterior ischemic optic neuropathy (AION) to those without giant cell arteritis (GCA). • Male sex is associated with AION despite GCA being more common in women. • Older age and higher CRP are associated with AION. • GCA patients with AION had a higher halo score than GCA patients without AION.

Headache-Related Characteristics of Biopsy-Confirmed Giant Cell Arteritis and the Relationship of Transmural Inflammation With Artery Tenderness and Chordal Thickening.

Giant cell arteritis (GCA) is characterized by headaches, but few studies have examined the detailed characteristics of pathologically confirmed cases. We investigated the characteristics of GCA patients, particularly headache, and their correlation with pathological findings. We retrospectively analyzed 26 patients (median age: 77.5 years, male: 38.4%) with GCA who underwent superficial temporal artery (STA) biopsy at the Japanese Red Cross Shizuoka Hospital between May 2001 and February 2022. All patients fulfilled the American College of Rheumatology and European League Against Rheumatism classification criteria for GCA. We focused on the relationship between clinical features, especially headache, and pathological findings. Twenty-four patients had unilateral, nonpulsatile, intermittent headaches. Transmural inflammation (TMI), a characteristic pathology of GCA, was present in 14 patients. Bivariate analysis revealed significant associations between the TMI and STA-related tenderness (odds ratio [OR]=11, 95% confidence interval [CI]=1.14 to 106.43, p=0.046) and the TMI and STA-related chordal thickening (OR=0.19, 95% CI=0.068 to 0.52, p=0.021). Headache in GCA patients was often unilateral, nonpulsatile, and intermittent. This study highlights the significant association of TMI with STA tenderness and ligamentous thickening, which has not been reported previously. Abnormal STA findings were significantly associated with pathological changes in GCA patients, emphasizing the importance of these lesions in predicting GCA.

Evaluation of baseline F-18 FDG positron emission tomography in the diagnosis and assessment of giant cell arteritis.

The aim of this study is to evaluate the baseline F18-FDG PET/CT findings of individuals diagnosed with giant cell arteritis (GCA) and to explore its association with clinical findings and classification criteria. We analysed data from patients who underwent F18-FDG PET/CT scans to investigate large vessel (LV) involvement between 2010 and 2019. Only patients with a clinical diagnosis of GCA and at least 6 months of follow-up were included. We compared initial clinical features and laboratory findings based on the presence of LV vasculitis on PET/CT and the maximum standard uptake value (SUVmax) of vascular territories. Twenty-nine patients (median age at diagnosis: 70, F/M: 24/5) were included in the study. Among them, 21 patients (72.4%) presented with cranial symptoms, while 8 patients (27.5%) had isolated LV-GCA. Twenty-two patients (75.9%) met the ACR/EULAR 2022 GCA classification criteria. LV vasculitis was detected on PET/CT in 23 patients (79.3%). A positive correlation was observed between SUVmax in the thoracic aorta and both CRP and ESR levels (r = 0.50, p = 0.026 and r = 0.63, p = 0.002, respectively). PET/CT positive patients were found to be younger (p = 0.016) and more frequently female (p = 0.017). They also exhibited fewer headaches (56.5% vs. 100%, p = 0.04), experienced fewer flares during follow-up (p = 0.03), and had a lower cumulative glucocorticoid dose at the 6th month (p = 0.036). Comparison of PET/CT-positive patients (n = 23) based on the fulfilment of the ACR/EULAR 2022 classification criteria revealed that patients who met these criteria were older (p = 0.02) and had significantly lower CRP levels at diagnosis (p = 0.02). The performance of F18-FDG PET/CT in diagnosing LV involvement in GCA is favourable, and the severity of FDG uptake in the vessel wall correlates with the acute phase response. Patients with extracranial involvement on PET/CT exhibit distinct features, including a younger age and female predominance. Additionally, these patients appear to experience fewer relapses and require lower doses of glucocorticoids. However, the clinical significance of PET/CT in patients who met ACR/EULAR classification criteria, predominantly consisting of patients with ischemic cranial symptoms, could not be determined in our study.

Clinical phenotype and complications of large vessel giant cell arteritis: A systematic review and meta-analysis.

Giant Cell Arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large vessel (LV) involvement, studies reporting incidence, clinical characteristics and complications of large-vessel GCA (LV-GCA) show conflicting results due to inconsistent disease definitions, differences in study methodologies and the broad spectrum of clinical presentations. The aim of this systematic literature review was to better define LV-GCA based on the available literature and identify distinguishing characteristics that may differentiate LV-GCA patients from those with limited cranial disease. Published studies indexed in MEDLINE and EMBASE were searched from database inception to 7th May 2021. Studies were included if they presented cohort or cross-sectional data on a minimum of 25 patients with LV-GCA. Control groups were included if data was available on patients with limited cranial GCA (C-GCA). Data was quantitatively synthesised with application of a random effects meta-regression model, using Stata. The search yielded 3488 studies, of which 46 were included. Diagnostic criteria for LV-GCA differed between papers, but was typically dependent on imaging or histopathology. Patients with LV-GCA were generally younger at diagnosis compared to C-GCA patients (mean age difference -4.53years), had longer delay to diagnosis (mean difference 3.03months) and lower rates of positive temporal artery biopsy (OR: 0.52 [95% CI: 0.3, 0.91]). Fewer LV-GCA patients presented with cranial manifestations and only 53% met the 1990 ACR Classification Criteria for GCA. Vasculitis was detected most commonly in the thoracic aorta, followed by the subclavian, brachiocephalic trunk and axillary arteries. The mean cumulative prednisolone dose at 12-months was 6056.5mg for LV-GCA patients, relapse rates were similar between LV- and C-GCA patients, and 12% of deaths in LV-GCA patients could be directly attributed to an LV complication. Patients with LV-GCA have distinct disease features when compared to C-GCA, and this has implications on diagnosis, treatment strategies and surveillance of long-term sequalae.

2022 American College of Rheumatology/EULAR Classification Criteria for Giant Cell Arteritis.

To develop and validate updated classification criteria for giant cell arteritis (GCA). Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate items, 2) prospective collection of candidate items present at the time of diagnosis, 3) expert panel review of cases, 4) data-driven reduction of candidate items, 5) derivation of a points-based risk classification score in a development data set, and 6) validation in an independent data set. The development data set consisted of 518 cases of GCA and 536 comparators. The validation data set consisted of 238 cases of GCA and 213 comparators. Age ≥50 years at diagnosis was an absolute requirement for classification. The final criteria items and weights were as follows: positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5); erythrocyte sedimentation rate ≥50 mm/hour or C-reactive protein ≥10 mg/liter (+3); sudden visual loss (+3); morning stiffness in shoulders or neck, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality on vascular examination, bilateral axillary involvement on imaging, and fluorodeoxyglucose-positron emission tomography activity throughout the aorta (+2 each). A patient could be classified as having GCA with a cumulative score of ≥6 points. When these criteria were tested in the validation data set, the model area under the curve was 0.91 (95% confidence interval [95% CI] 0.88-0.94) with a sensitivity of 87.0% (95% CI 82.0-91.0%) and specificity of 94.8% (95% CI 91.0-97.4%). The 2022 American College of Rheumatology/EULAR GCA classification criteria are now validated for use in clinical research.

Prevalence and significance of ischemic cerebrovascular events in giant cell arteritis

ObjectivesTo determine the prevalence of cerebrovascular events (CVE) in giant cell arteritis (GCA) and to alert clinicians to the importance of early detection of CVE in this disease. MethodsRetrospective observational study involving a cohort of GCA patients. Demographic, clinical and laboratory data were collected. All patients fulfilled the American College of Rheumatology (ACR) 1990 GCA classification criteria and had a positive ultrasound test for GCA in agreement with the EULAR recommendations. Demographic and clinical parameters were recorded with special attention paid to ischemic cranial events. ResultsWe studied 123 consecutive GCA patients, 74 (60.2%) women with a mean age of 79 years. Twelve patients (9.75%) suffered from neurologic symptoms other than AION, of whom 9 (7.3%) experienced ischemic events related to GCA and 3 (2.44%) likely experienced CVE due to other common causes. Of the 9 patients with CVE caused by GCA, 5 were diagnosed with transient ischemic attacks (TIAs), 2 with ischemic stroke, and 2 were cases involving cranial nerve palsies. High rates of mortality were found in patients with a TIA or stroke, while polymyalgia rheumatica (PMR) appeared to confer some protection against ischemic pathologies in GCA patients. ConclusionsStroke and TIA are common presentation patterns associated with GCA and should be suspected in all CVE-related cases with high acute-phase reactants commonly present in the elderly. This ischemic subgroup exhibited a higher mortality rate.

Temporal artery biopsy for suspected giant cell arteritis: a retrospective analysis.

Temporal artery biopsy (TAB) is one of the diagnostic criteria of giant cell arteritis (GCA) according to 1990 ACR criteria and remains a tool for diagnosis. Although clinicians perform TAB with an intent to confirm suspected GCA, some biopsies result in negative and some lead to non-GCA diagnoses. We aim to review the diagnoses after TAB biopsy performed for suspected GCA and also wanted to evaluate the diagnostic changes and concomitant diseases that develop over time. The patients who had undergone TAB for suspected GCA were identified using the record entry code for TAB. Patients meeting the classification criteria for GCA were designated as the GCA group and not meeting criteria were designated as a non-GCA group. Other classification criteria were implemented for the non-GCA group diseases. A total of 51 patients (Female: 62.7%, median age: 72.1 ± 7.4years) who had undergone TAB for suspected GCA were evaluated. TAB was positive in 23 (69.6%) of the 33 patients who met the GCA classification criteria. No significant difference was found between TAB-positive and TAB-negative GCA patients in terms of clinical and laboratory parameters. In the non-GCA group, 12 patients had isolated polymyalgia rheumatica (PMR), and the diagnoses of the remaining six patients were as follows: four large vessel vasculitis (LVV) not satisfying GCA diagnostic criteria, one chronic myelomonocytic leukemia (CMML), and one amyloidosis. TAB was negative in all patients with isolated PMR. TAB showed primary amyloidosis in one patient. Out of 33 GCA patients, 21 had "isolated" GCA, four had GCA + Rheumatoid arthritis (RA), seven had GCA + PMR, and one had GCA + polymyositis. RA was diagnosed antecedent to GCA in two patients, and after GCA in the other two patients. One of the patients had developed GCA 20years after polymyositis had been diagnosed. TAB was found to be positive in two-thirds of patients with suspected GCA. Late-onset RA and rarely other inflammatory rheumatic diseases may develop in the course of GCA.

Prevalence and significance of ischemic cerebrovascular events in giant cell arteritis

ObjectivesTo determine the prevalence of cerebrovascular events (CVE) in giant cell arteritis (GCA) and to alert clinicians to the importance of early detection of CVE in this disease. MethodsRetrospective observational study involving a cohort of GCA patients. Demographic, clinical and laboratory data were collected. All patients fulfilled the American College of Rheumatology (ACR) 1990 GCA classification criteria and had a positive ultrasound test for GCA in agreement with the EULAR recommendations. Demographic and clinical parameters were recorded with special attention paid to ischemic cranial events. ResultsWe studied 123 consecutive GCA patients, 74 (60.2%) women with a mean age of 79 years. Twelve patients (9.75%) suffered from neurologic symptoms other than AION, of whom 9 (7.3%) experienced ischemic events related to GCA and 3 (2.44%) likely experienced CVE due to other common causes. Of the 9 patients with CVE caused by GCA, 5 were diagnosed with transient ischemic attacks (TIAs), 2 with ischemic stroke, and 2 were cases involving cranial nerve palsies. High rates of mortality were found in patients with a TIA or stroke, while polymyalgia rheumatica (PMR) appeared to confer some protection against ischemic pathologies in GCA patients. ConclusionsStroke and TIA are common presentation patterns associated with GCA and should be suspected in all CVE-related cases with high acute-phase reactants commonly present in the elderly. This ischemic subgroup exhibited a higher mortality rate.

OP0144 EFFECT OF TOCILIZUMAB ON VASCULAR INFLAMMATION BY 18F-FLUORODEOXYGLUCOSE POSITRON EMISSION TOMOGRAPHY: A PROSPECTIVE, LONGITUDINAL STUDY

Background:Two randomized controlled trials have demonstrated clinical efficacy of tocilizumab for the treatment of giant cell arteritis (GCA)(1, 2). In these trials, clinical and laboratory measures were used to define the outcome measures. The direct effect of tocilizumab on vascular inflammation remains poorly characterized.Objectives:To prospectively evaluate vascular inflammation as measured by18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) in a longitudinal cohort of patients with GCA treated with tocilizumab over a several year follow-up period.Methods:Patients with GCA were recruited into a prospective, observational cohort. All patients fulfilled modified 1990 American College of Rheumatology (ACR) Classification Criteria for GCA. All patients underwent FDG-PET computed tomography (CT) prior to initiation of tocilizumab. A single reader reviewed all PET scans, blinded to clinical data. Qualitative assessment of FDG uptake relative to liver uptake by visual assessment (scale 0-3) was assessed in 9 arterial territories. A summary score, PET Vascular Activity Score (PETVAS), was calculated (scale 0-27).Patients underwent imaging at 6-12 month intervals per a standardized imaging protocol. In a subset of patients in whom tocilizumab was discontinued due to established remission, a repeat FDG-PET scan was obtained within 6 months of drug discontinuation.Change in PET activity over time was measured by linear regression. PET activity during established remission was compared to PET activity after discontinuation of tocilizumab. For some patients, tocilizumab was added to the existing treatment regimen without a substantive change in concomitant glucocorticoid dose. In a secondary analysis, patients were stratified by prednisone dosing (high dose prednisone &gt;10mg/day prednisone, low dose prednisone ≤10mg/day prednisone during the imaging interval) to determine if tocilizumab had an effect on vascular inflammation independent of glucocorticoids.Results:22 patients were included in the study. All patients had clinically active disease at baseline with median baseline PETVAS 24.5 (23-27). There was a significant reduction in PETVAS over 2 years follow up (p&lt;0.01 for linear trend) (Figure). Of note, there was continued progressive improvement in PETVAS in both year 1 and year 2 of treatment. Eight patients received concomitant high dose glucocorticoids and 14 patients remained on low dose glucocorticoids with the addition of tocilizumab. In patients who only received low dose prednisone, significant reduction in PETVAS was still observed with addition of tocilizumab (PETVAS 25.5 to 19.5, p=0.04). In a subset of 5 patients who discontinued tocilizumab due to established remission [median PETVAS 19 (17.5-22) at time of remission], a repeat FDG-PET scan within 6 months after treatment discontinuation showed worsening PET activity in 4 out of 5 patients [median PETVAS 23 (20-23)]. Two of these patients subsequently experienced a clinical disease relapse.Conclusion:Tocilizumab was associated with improved vascular inflammation as assessed by FDG-PET. There was continued improvement of vascular inflammation at both year 1 and year 2 of treatment, and early evidence suggests a rebound of vascular inflammation when tocilizumab was discontinued. These data provide rationale for long-term tocilizumab therapy in patients with GCA and for consideration of FDG-PET as an outcome measure in future clinical trials.

FRI0197 THE DIAGNOSTIC ACCURACY OF VASCULAR ULTRASOUND IN PATIENTS SUSPECTED OF GCA: A DANISH MULTICENTRE PROSPECTIVE COHORT STUDY

Background:Giant Cell Arteritis (GCA) is one of the most common systemic vasculitis. Temporal artery biopsy (TAB) has been the standard test to confirm the diagnosis of GCA. However, TAB has a lower sensitivity than clinical diagnosis and up to 44% of biopsy-negative patients are clinically diagnosed as having GCA.In a recent meta-analysis of the diagnostic performance of ultrasound (US) in GCA the sensitivity was 77 % (1). The included studies were performed by expert groups in single centres. In the to date only multicentre study (TABUL) investigating the diagnostic accuracy of US compared to clinical diagnosis after 6 months the sensitivity was lower (54%) (2)Objectives:To evaluate the diagnostic accuracy of vascular US compared to TAB in a multicentre study.Methods:In three Danish centres patients suspected for GCA were included during a period of two years. At baseline, clinical and laboratory data were collected and vascular US of temporal, facial, common carotid and axillary artery were performed. The US examinations were performed with high frequency transducers (15-18 MHZ) and followed by a TAB. All ultrasongraphers had participated in the same standardized US educational program and were blinded to clinical and laboratory data. An external expert blinded to clinical and laboratory data evaluated all images and made the final US diagnosis.A positive sign for vasculitis in cranial arteries was defined as a hypoechoic intima media complex (IMC) thickening (halo sign) and a positive compression sign. A homogeneous IMC increased thickness in axillary artery of ≥1mm and in common carotid artery ≥1.5mm was defined as vasculitis.The consultant rheumatologist’s diagnosis at 6 months after initial presentation was considered as the reference standard for the diagnosis of GCA.Results:During the recruitment period, 112 patients were included, 59% females, mean (SD) age 72.4(7.9) years, among which 91(81.3%) fulfilled the ACR 1990 classification criteria for GCA. 92% of the patients reported a newly emerged localized headache, while 49 (43.8%) experienced polymyalgia rheumatic symptoms.TAB was positive in 46(41.1%) and inconclusive in 6 patients, who were excluded from the analysis. Mean (SD) duration of glucocorticoid therapy prior to US and TAB was 0.91(1.55) and 4.02(2.61) days, respectively. In 62 patients, the final diagnosis was GCA.In all patients with a positive TAB, the US of the temporal artery was also positive for GCA. Of 19 cases with positive US and negative TAB, 12 were clinically diagnosed with GCA of whom 6 had isolated large vessel involvement on US. Among 41 patients with both negative US and TAB, 4 were clinically diagnosed with GCA (Box 1)US had a sensitivity of 93% and specificity of 84% for the diagnosis of GCA, while the sensitivity for TAB was lower (74%) with a specificity of 100%. For the diagnosis of GCA, US had a PPV of 89.2 % and a NPV of 90.2%, while for TAB the PPV was 100% and the NPV 73.3%.Conclusion:US evaluation of the temporal, facial and selected supraaortic arteries performed by trained ultrasonographers can replace biopsy in the diagnosis of GCA.Box.1

Multivariable prediction model for suspected giant cell arteritis: development and validation.

PurposeTo develop and validate a diagnostic prediction model for patients with suspected giant cell arteritis (GCA).MethodsA retrospective review of records of consecutive adult patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at seven university centers. The pathologic diagnosis was considered the final diagnosis. The predictor variables were age, gender, new onset headache, clinical temporal artery abnormality, jaw claudication, ischemic vision loss (VL), diplopia, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelet level. Multiple imputation was performed for missing data. Logistic regression was used to compare our models with the non-histologic American College of Rheumatology (ACR) GCA classification criteria. Internal validation was performed with 10-fold cross validation and bootstrap techniques. External validation was performed by geographic site.ResultsThere were 530 complete TABx records: 397 were negative and 133 positive for GCA. Age, jaw claudication, VL, platelets, and log CRP were statistically significant predictors of positive TABx, whereas ESR, gender, headache, and temporal artery abnormality were not. The parsimonious model had a cross-validated bootstrap area under the receiver operating characteristic curve (AUROC) of 0.810 (95% CI =0.766–0.854), geographic external validation AUROC’s in the range of 0.75–0.85, calibration pH–L of 0.812, sensitivity of 43.6%, and specificity of 95.2%, which outperformed the ACR criteria.ConclusionOur prediction rule with calculator and nomogram aids in the triage of patients with suspected GCA and may decrease the need for TABx in select low-score at-risk subjects. However, misclassification remains a concern.

Histopathologic Findings of Patients With Biopsy-Negative Giant Cell Arteritis Compared to Those Without Arteritis: A Population-Based Study.

To evaluate whether there are histopathologic features of negative temporal artery biopsy (TAB) that allow differentiation between patients with giant cell arteritis (GCA) and those without. All consecutive patients with negative TAB seen between January 2009 and December 2012 were selected retrospectively. Demographic, clinical, and laboratory data at presentation and at each followup visit were collected. A pathologist with expertise in vasculitis and blinded to clinical data and final diagnosis reviewed all negative TABs. Histopathologic features evaluated were the presence of a focal mediointimal scar, medial attenuation, intimal hyperplasia, fragmentation of inner elastic lamina, calcification, adventitial fibrosis, and neoangiogenesis. After a median (interquartile range [IQR]) followup period of, respectively, 19 months (9.2-31.2) and 26.3 months (4.9-36.7) (P = 0.041), 38 (55%) of 69 patients had a final diagnosis of TAB-negative GCA, while in the remaining 31 (45%) of 69 patients GCA was excluded. The American College of Rheumatology classification criteria for GCA were satisfied by 31 (81.6%) of 38 patients and 2 (6.5%) of 31 patients (P < 0.0001). Compared to non-GCA patients, those with TAB-negative GCA had more frequent cranial manifestations and higher erythrocyte sedimentation rates and C-reactive protein levels. The frequency of patients receiving prednisone, the mean dose, and the duration of prednisone treatment at TAB were similar in the 2 groups. There were no significant differences between TAB-negative GCA and non-GCA patients in the frequencies of all the histologic features evaluated. The histologic features of negative TABs evaluated in this study do not allow for the differentiation between GCA and non-GCA patients. In the absence of an inflammatory infiltrate, other histologic changes of the temporal artery wall are not specific for GCA.

Giant Cell Arteritis: Read the Fine Print!

Giant Cell Arteritis: Read the Fine Print!

FRI0464 Development of the Classification Criteria for GIANT Cell Arteritis in the Diagnostic and Classification Criteria for Vasculitis Study: A Pilot Study Using A Panel Review Methodology

BackgroundThe Diagnostic and Classification Criteria for Vasculitis (DCVAS) Study is a multinational observational study to develop diagnostic criteria and to update classification criteria for the primary systemic vasculitides. By 2015...

FRI0471 Negative Temporal Artery Biopsies: Pathologic Findings of Patients with Biopsy-Negative GIANT Cell Arteritis Compared to Those of Patients without Arteritis

BackgroundTemporal artery biopsy (TAB) showing transmural inflammation is considered the gold standard for the diagnosis of giant cell arteritis (GCA). A negative TAB does not rule out GCA, and a...

OP0206 Diagnostic Performance of PET/CT in Patients with Newly Diagnosed, Biopsy-Proven, Giant-Cell Arteritis. a Prospective, Case-Control Study Using Roc Analysis at Different Vascular Territories

BackgroundPET has emerged as a promising diagnostic tool for the detection of large-vessel involvement in giant cell arteritis (GCA). In a recent meta-analysis including some heterogeneous studies, PET sensitivity and...

OP0207 The “Fast Track” Giant Cell Arteritis Outpatient Clinic: A Useful Tool to Reduce the Morbidity of Disease?

BackgroundPermanent visual loss is one of the most serious complications in giant cell arteritis (GCA). Visual manifestations have been reported in 26% and permanent visual loss in up to 15%...

FRI0219 Involvement of supra-aortic vessels in giant cell arteritis diagnosed by color duplex ultrasonography

BackgroundThe highest incidence of giant cell arteritis (GCA) worldwide has been reported from Southern Norway [1]. Aortic branch involvement [i.e. large vessel vasculitis (LVV)] has been reported to be present...

Web of Confusion

c t p m ( PRESENTATION The occurrence of a late-stage complication in a patient with early disease, reminds us that any illness can take an unpredictable course. A 63-year-old man presented with complaints of fatigue and rash. He had been in his usual state of health until approximately 2 months prior, when he developed decreased visual acuity in his right eye. He was seen by a local ophthalmologist, who diagnosed nonarteritic ischemic optic neuritis and prescribed a daily aspirin. Soon thereafter, the patient noticed that he was having trouble moving about his house and working, and he felt as if he needed to sit and rest often. Additionally, he admitted to night sweats and a 15-lb weight loss. Upon questioning, he also reported that he had developed a nonpruritic rash on his legs and torso. He denied headache, joint pain, myalgia, morning stiffness, jaw claudication, weakness, cough, or shortness of breath. He took no medications other than a daily adult aspirin. On examination, the patient exhibited livedo reticularis involving his lower limbs, buttocks, and lower torso circumferentially (Figures 1 and 2). He was normotensive, afebrile, and had no other rash, nodules or ulcers. His joints were normal, as were his heart, lungs, and abdomen. Peripheral pulses were present, and his temporal arteries appeared normal and were nontender.

The diagnostic value of ultrasonography-derived edema of the temporal artery wall in giant cell arteritis: a second meta-analysis

BackgroundUltrasonography of temporal arteries is not commonly used in the approach of patients with suspected giant cell arteritis (GCA) in clinical practice. A meta-analysis of primary studies available through April 2004 concluded that ultrasonography could indeed be helpful in diagnosing GCA. We specifically re-examined the diagnostic value of the ultrasonography-derived halo sign, a dark hypoechoic circumferential thickening around the artery lumen, indicating vasculitic wall edema, in GCA.MethodsOriginal, prospective studies in patients with suspected GCA that examined ultrasonography findings of temporal arteries using the ACR 1990 classification criteria for GCA as reference standard, published through 2009, were identified. Only eight studies involving 575 patients, 204 of whom received the final diagnosis of GCA, fulfilled technical quality criteria for ultrasound. Weighted sensitivity and specificity estimates of the halo sign were assessed, their possible heterogeneity was investigated and pooled diagnostic odds ratio was determined.ResultsUnilateral halo sign achieved an overall sensitivity of 68% (95% CI, 0.61-0.74) and specificity of 91% (95% CI, 0.88-0.94) for GCA. The values of inconsistency coefficient (I2) of both sensitivity and specificity of the halo sign, showed significant heterogeneity concerning the results between studies. Pooled diagnostic odds ratio, expressing how much greater the odds of having GCA are for patients with halo sign than for those without, was 34 (95% CI, 8.21-138.23). Diagnostic odds ratio was further increased to 65 (95% CI, 17.86-236.82) when bilateral halo signs were present (sensitivity/specificity of 43% and 100%, respectively). In both cases, it was found that DOR was constant across studies.ConclusionTemporal artery edema demonstrated as halo sign should be always looked for in ultrasonography when GCA is suspected. Providing that currently accepted technical quality criteria are fulfilled, halo sign's sensitivity and specificity are comparable to those of autoantibodies used as diagnostic tests in rheumatology. Validation of revised GCA classification criteria which will include the halo sign may be warranted.