Abnormal Temporal Artery Research Articles

Upper respiratory tract and orofacial manifestations of new-onset giant cell arteritis: results from a large, prospective inception cohort study.

Giant cell arteritis (GCA) often features upper respiratory tract (URT) and orofacial manifestations, which signal the involvement of external carotid artery branches. In this study, we aimed to describe the frequency of various URT/orofacial symptoms at GCA onset, as well as the main characteristics of patients presenting these symptoms. We included all patients who were newly diagnosed with GCA between 1976 and April 2022 at the Internal Medicine Department of a tertiary-care hospital. Ten URT or orofacial symptoms were prospectively examined systematically in each patient. We used multivariate analyses to identify the GCA characteristics, including URT/orofacial symptoms, associated with temporal artery biopsy (TAB) positivity. At least 1 URT/orofacial symptom was present in 68.6% of the 599 patients (3 or more symptoms in 30% of cases). Jaw claudication, maxillary pain, and pain during mouth opening were the most prevalent symptoms. Dry cough was recorded in 17% of cases. GCA patients with URT/orofacial symptoms had more clinical abnormalities of the temporal artery bed and ischaemic ophthalmological complications, but less large-vessel vasculitis according to imaging. The likelihood of a positive TAB was increased in patients with an abnormal temporal artery upon clinical examination (OR 4.16; CI 2.75-6.37, p < 0.001) or jaw claudication (OR 2.18; CI 1.35-3.65, p = 0.002), and decreased in those with hoarseness (OR 0.47; CI 0.26-0.87, p = 0.02) or earache (OR 0.54; CI 0.31-0.95, p = 0.03). Isolated URT/orofacial presentation (i.e., without headache or visual signs) accounted for 5.2% of the entire cohort. Oral-facial symptoms were present in two-thirds of GCS cases. Thus, they could serve as leading clinical clues for a GCA diagnosis, and are a risk factor for permanent visual loss. Several URT/orofacial symptoms such as jaw claudication, hoarseness, and earache influenced the likelihood of a positive TAB. Isolated URT/orofacial presentation of GCA is a rare but potentially challenging occurrence.

Giant Cell Arteritis of the Superior Mesenteric Artery Presenting With Wernicke Encephalopathy From Thiamine Deficiency

Objective N/A. Background Giant cell arteritis (GCA) is one of the most common systemic vasculitides in adults over the age of 50 with incidence ranging from 15 to 35 per 100,000 individuals. The disorder is often included in the differential diagnosis of maladies producing atypical facial pain, headache, visual loss, amaurosis fugax, jaw pain, elevated inflammatory markers, and anemia. GCA is typically known to affect cranial arteries with physical exam findings of tenderness to palpation of the temporal arteries and cranial neuropathies. Clinical diagnosis is further supported by new headache, temporal artery abnormality, elevated ESR (= 50 mm/h), and abnormal artery biopsy. Design/Methods N/A. Results A 68-year-old female with history of primary generalized seizures presented to clinic with a 6-week history of paroxysms of acute confusional episodes, the inability to arise from a seated position due to lower extremity weakness bilaterally, alterations of consciousness without loss of consciousness, severe anorexia, and weight loss. MRI with contrast including Axial FLAIR/T2/Diffusion revealed bilateral pan-lobar cortical and subcortical atrophy with ex-vacuo ventriculomegaly and mild leukoaraiosis in the subcortical white matter tracts. PET-CT body revealed linear uptake involving the aortic root, extending into subclavian arteries bilaterally with segmental involvement of proximal common carotids, and extending inferiorly to the level of the common iliac arteries and the mesenteric arteries. Temporal artery biopsy revealed presence of granulomas with multinucleated giant cells. Serology panel revealed pan hypovitaminoses in Vitamins A, B1, B6, B12, and D. Conclusions Traditional GCA workup initially resulted inconclusive for the patient, whose condition deteriorated as the patient's altered mental status and dizziness spells continued unremittingly. This case highlights the link between large vessel vasculitis and malabsorption syndromes, with the involvement of the superior mesenteric artery, a medium sized vessel, in GCA previously unrecognized. Furthermore, this case is a superb example of multiple etiologies of treatable causes of reversible dementia.

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.

Case Report: Giant Cell Arteritis presenting with 6th Nerve Palsy without Ischemic Optic Neuropathy

Purpose: Giant cell arteritis (GCA) can be a difficult condition to identify in the early stages especially in the absence of the pathognomonic arteritic anterior ischemic optic neuropathy. Optometrists serve an important role in correctly triaging and initiating appropriate work-up and treatment for this emergent condition. This case report and review serves as a refresher of the systemic and ocular signs and symptoms of GCA. Background: GCA is a systemic autoimmune condition characterized by granulomatous inflammation of medium and large arteries in patients over 50. The most well-known constellation of signs and symptoms include new onset headache, jaw claudication, scalp tenderness, temporal artery abnormality with painless vision loss secondary to arteritic anterior ischemic optic neuropathy. However, it is important to be aware of alternate presenting signs and symptoms including pain anywhere in the distribution of the external carotid (occipital, neck, tongue, throat, ear) and signs of large vessel GCA (arm/limb claudication, chest/back pain, Raynaud’s phenomenon) and Polymyalgia Rheumatica (PMR) (bilateral hip/shoulder pain and morning stiffness). Case Report: An 86-year-old Caucasian female presents for a referred exam regarding new onset diplopia with concurrent occipital headache, neck pain and sore throat originally dismissed as symptoms of her other systemic health conditions. ESR, CRP and platelets were elevated on serology and subsequent temporal artery biopsy was positive for GCA. Oral steroids were initiated, and she was lost to follow up after her ocular symptoms resolved. Conclusion: GCA can present with a large range of manifestations, and many are nonspecific and easily attributable to other causes especially when it deviates from the classic constellation of new onset headache, jaw claudication, scalp tenderness and temporal artery abnormality with painless vision loss. Optometrists as primary eye care providers may be the first point of contact and need to be cognizant of the broader set of manifestations to minimize delays in diagnosis and treatment of this life and vision threatening condition. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-report-giant-cell-arteritis-presenting-with-6th-nerve-palsy-without-ischemic-optic-neuropathy) Please check COPE course expiry date prior to enrollment. The COPE course test must be taken before the course expiry date.

Features and risk factors for new (secondary) permanent visual involvement in giant cell arteritis.

New permanent visual loss (PVL) in treated patients with giant cell arteritis (GCA) is a rare but worrisome occurrence. In this study, we aimed to describe the frequency and main features of new PVL occurring after the beginning of glucocorticoid therapy in patients with newly diagnosed GCA. We included in an inception cohort all consecutive patients newly diagnosed with GCA in the internal medicine department of a tertiary-care hospital between 1976 and May 2020. The study population comprised all the patients without bilateral PVL before treatment who were followed for at least one year. Only well-documented visual events that set after the initiation of glucocorticoid treatment were regarded as new PVL. Eleven out of 502 patients (2.2%) experienced a new PVL including 6 occurrences during the initial therapeutic phase and 5 during the tapering phase. Patients with new PVL during treatment had higher mean age, more often displayed temporal artery abnormalities on physical examination, and had higher mean platelet counts at GCA onset. There was a strong excess risk of contralateral recurrence during treatment in patients with unilateral loss at GCA onset compared with patients with uncomplicated GCA (10.5% vs 1.1%, OR=10.26, p<0.001). New PVL in treated GCA is a rare, but significant occurrence. Older patients and patients who already had unilateral PVL at diagnosis have higher risk of new ischaemic visual loss during treatment compared to the other patients. Close clinical, laboratory, and eye monitoring of these high-risk patients is of paramount importance.

P206 Rethinking old biomarkers: novel predictors of giant cell arteritis

Abstract Background/Aims Giant cell arteritis (GCA) is a large vessel vasculitis mainly affecting the arteries of the head and neck which, if untreated, may lead to permanent vision loss. Glucocorticoids are highly effective at turning off inflammation but come with toxic side effects, making prompt diagnosis essential. There is no gold-standard investigation for GCA. Although specific, temporal artery biopsy (TAB) is only positive in approximately 25% of cases making it problematic as a rule-in diagnostic test. Vascular ultrasound may aid diagnosis but there is a rapid reduction in sensitivity with glucocorticoid use and it is not yet universally available. Diagnosis therefore requires the integration of clinical judgment with blood tests measuring inflammation, imaging, and biopsy. Aims:Identify which components of the history, examination and laboratory findings are most predictive of a positive diagnosis of GCA in the local region of Yeovil District Hospital, and to investigate the usefulness of alternative blood biomarkers. Methods Data was collected from GCA clinic attendances between August 2018 and February 2020 using electronic notes, clinic letters and the pathology system. Predictive values, sensitivity, specificity, and receiver operating characteristic (ROC) curves were calculated for each individual parameter and for groups of parameters. Results Ninety-one patients presented to GCA clinic in the 18 months studied. Median age was 71 and 73% were female. 56 patients with suspected disease went on to have TAB, of which 38/56 (68%) were of adequate length ( &amp;gt; =10mm), and of those, 12/38 (32%) confirmed a diagnosis of GCA. 43/91 (47%) patients were ultimately diagnosed biopsy proven or suspected GCA. Headache was the most common presenting feature (88%) followed by raised ESR (55%), raised CRP (53%), visual disturbance (44%), scalp tenderness (33%), jaw claudication (31%), PMR symptoms (27%) and temporal artery abnormalities (20%). Headache and raised CRP+/-ESR were the most sensitive markers (91% and 100%, respectively). They were, however, the least specific (4% and 36%). Temporal artery abnormality was the most specific finding (81%). ROC analysis revealed that the best-performing biomarkers were monocytes (area under the ROC curve (AUC) of 0.81) and platelets (AUC 0.80), which were superior to jaw claudication, the best-performing classical biomarker (AUC 0.68). Platelets above 450 x 109/L had a specificity of 96% with a likelihood ratio of 10.9. Monocytes above 0.45 x 106/L had sensitivity and specificity of 100% and 67%, respectively. Conclusion GCA cannot be accurately predicted by any single feature. In this cohort, absence of headache with a normal CRP+/-ESR ruled out GCA. Platelets and monocytes performed better than all the classical parameters associated with GCA. Validation of these biomarkers in a larger cohort is now needed to ascertain cut-off points which may help to develop a more accurate method to predict cases of GCA. Disclosure T. Somoskeoy: None. A. Bourn: None. S. Knights: None. B. Mulhearn: None.

Associations among temporal and large artery abnormalities on vascular ultrasound in giant cell arteritis

Objectives: Giant cell arteritis (GCA) can manifest in cranial and/or extracranial arteries. We investigated the distribution of affected arteries on vascular ultrasound (VUS) among patients with new-onset or prior-onset GCA. Method: We retrospectively studied patients with either new-onset or prior-onset GCA and an abnormal VUS, from 2013 to 2017. Trained vascular technologists imaged the bilateral temporal arteries and carotid, axillary, and subclavian arteries. Vascular medicine physicians interpreted the images. Vasculitis-related abnormalities in individual vessels and their distribution (temporal artery, large artery, or both) were evaluated. Phi coefficients (φ) and Fisher’s exact test were used to assess correlations among individual abnormal arteries. Results: Among 66 GCA patients, 28.8% had prior-onset GCA (median duration 17.8 months). Acute arteritis on VUS was observed in the majority of patients with both new-onset (72.3%) and prior-onset GCA (68.4%); the remainder had hyperechoic wall thickening without acute arteritis. Involvement of the temporal arteries only (45.5%) or large arteries only (34.8%) was more common than involvement of both (19.7%); this finding was similar in new-onset and prior-onset GCA. There were moderate positive correlations among temporal artery branches (φ = 0.51–0.58, p < 0.003) and among axillary and subclavian arteries (φ = 0.51–0.77, p < 0.003), and moderate negative correlations between abnormalities in the temporal and large arteries (φ = −0.46 to −0.58, p < 0.003). Conclusion: On VUS, vasculitis-related abnormalities in the temporal arteries only or large arteries only were more common than concurrent temporal and large artery abnormalities in patients with both new-onset GCA and prior-onset GCA.

Temporal Arteritis Revealing Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A Case-Control Study.

Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are rarely revealed by TA manifestations, leading to a risk of misdiagnosis of GCA and inappropriate treatments. This study was undertaken to describe the clinical, biologic, and histologic presentations and outcomes in cases of TA revealing AAV (TA-AAV) compared to controls with classic GCA. In this retrospective case-control study, the characteristics of patients with TA-AAV were compared to those of control subjects with classic GCA. Log-rank test, with hazard ratios (HRs) and 95% confidence intervals (95% CIs), was used to assess the risk of treatment failure. Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three patients (66%) presented with atypical symptoms of GCA (ear, nose, and throat involvement in 32% of patients, and renal, pulmonary, and neurologic involvement in 26%, 20%, and 16% of patients, respectively). Blood samples were screened for ANCAs at the time of disease onset in 33 patients, and results were positive in 88%, leading to a diagnosis of early TA-AAV in 20 patients. The diagnosis of AAV was delayed a median interval of 15 months in 30 patients. Compared to controls with GCA, patients with TA-AAV were younger (median age 70 years versus 74 years), were more frequently men (48% versus 30%), and had high frequencies of atypical manifestations and higher C-reactive protein levels (median 10.8 mg/dl versus 7.0 mg/dl). In patients with TA-AAV, temporal artery biopsy (TAB) showed fibrinoid necrosis and small branch vasculitis in 23% of patients each, whereas neither of these characteristics was evident in controls with GCA. Treatment failure-free survival was comparable between early TA-AAV cases and GCA controls, whereas those with delayed TA-AAV had a significantly higher risk of treatment failure compared to controls (HR 3.85, 95% CI 1.97-7.51; P < 0.0001). TA-AAV should be considered diagnostically in cases of atypical manifestations of GCA, refractoriness to glucocorticoid treatment, or early relapse. Analysis of TAB specimens for the detection of small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCAs should be performed in cases of suspected GCA with atypical clinical features and/or evidence of temporal artery abnormalities on TAB.

Optimal temporal artery biopsy length for diagnosis of giant cell arteritis.

Giant cell arteritis is a major type of large vessel vasculitis.1 Abrupt-onset headache with or without scalp tenderness, temporal artery abnormalities, and claudication (in the jaw, tongue, and limbs) might be clinically prominent, accompanied by constitutional symptoms and, in about half of patients, polymyalgia rheumatica.2 Patients with giant cell arteritis show no specific laboratory features, but most have elevated acute phase reactants at disease onset. The 2018 update of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis advise a confirmatory test in all patients with suspected giant cell arteritis, either by imaging (ie, ultrasound, MRI, CT, or PET-CT) or histology (ie, temporal artery biopsy).

Giant cell arteritis and its mimics: A comparison of three patient cohorts

ObjectiveTo compare temporal artery biopsy (TAB)-positive giant cell arteritis (GCA) to TAB-negative GCA and patients with GCA mimics Methods: Patients diagnosed with TAB-positive and TAB-negative GCA between 1/1/1998 and 12/31/2013 wereretrospectively identified. These two groups were compared to a cohort of patients with TAB performed between 1/1/2009 and 12/31/2010 in which the TAB was negative and alternative diagnosis was provided after a minimum of 6-months of follow-up. Baseline characteristics were compared between groups using chi-square and rank sum tests. Results591 study subjects were identified (286 TAB-positive, 110 TAB-negative GCA and 195 TAB-negative GCA mimics) during the respective study periods. Compared to TAB-negative GCA, GCA mimics had similar rates of headache and vision loss but significantly less frequent jaw/limb claudication, arterial bruits and constitutional symptoms, as well as lower platelet levels. Compared to TAB-positive GCA patients, TAB-negative GCA were younger, had shorter time to diagnosis, met fewer 1990 ACR classification criteria and had lower frequencies of polymyalgia rheumatica, jaw claudication and temporal artery abnormalities; but, higher frequency of arm claudication and constitutional symptoms. Among 61 TAB-negative patients with advanced arterial imaging, 43 (69%) had at least one abnormality consistent with GCA. ConclusionConsideration of alternative diagnoses is requisite in evaluating patients with negative TAB. Advanced imaging assists in identifying occult large-vessel vasculitis and should be employed in all TAB-negative patients with suspicion for GCA.

AB0537 DIAGNOSTIC ACCURACY OF SYMPTOMS AND SIGNS FOR GIANT CELL ARTERITIS: SYSTEMATIC REVIEW AND META-ANALYSIS

Background:Making a correct diagnosis of giant cell arteritis (GCA) is critical given the potential complications of the disease and its therapy. Estimation of the clinical probability of GCA is challenging. Prediction models might be helpful, but have methodological and practical draw backs. One earlier meta-analysis described the diagnostic accuracy of symptoms and signs for a positive temporal artery biopsy [1]. In the latter study, the diagnostic accuracy of symptoms and signs might have been overestimated due to inclusion of case-control studies.Objectives:To evaluate the diagnostic accuracy of symptoms and signs for GCA.Methods:PubMed, EMBASE and the Cochrane Database were searched for relevant studies. Studies were eligible if: all patients were suspected of having GCA; either a temporal artery biopsy (TAB), imaging test, or clinical diagnosis was used as reference standard for GCA; a 2x2 table was available for at least one symptom, physical sign or routine laboratory test (i.e. the index tests). Case reports and case-control studies were excluded. The screening, full text review, quality assessment with QUADAS-2 tool and data extraction were performed by two investigators. Hierarchical logistic regression modelling provided the pooled estimates of likelihood ratios with their 95% confidence intervals. Likelihood ratios &lt;0.5 (i.e. making GCA less likely) or &gt;2.0 (i.e. making GCA more likely) were considered diagnostically relevant.Results:Out of 1359 reports screened, 59 studies were included in the study. These reports contained 13406 patients, including 3940 GCA patients. Most studies were retrospective, performed at tertiary centres and published after the prior meta-analysis [1]. TAB was the reference standard in 36 studies and a clinical diagnosis in 23 studies. Quality assessment revealed substantial risk of selection bias in the majority of studies. Studies using a clinical diagnosis were at risk of bias, as the reference standard might be influenced by the index tests (e.g. symptoms). Jaw claudication, limb claudication, temporal tenderness, temporal artery abnormalities, especially arterial thickening or loss of pulse, and anterior ischemic optic neuropathy provided a positive likelihood ratio &gt;2.0. None of the 19 symptoms and 7 physical signs evaluated, provided a negative likelihood ratio &lt;0.5. An ESR &gt;60mm/hr and platelet count &gt;400*109/L provided a positive likelihood ratio of &gt;2.0. Absence of an elevated CRP level or ESR &gt;60mm/hr yielded a negative likelihood ratio of &lt;0.5. Studies using a clinical diagnosis as reference standard reported higher positive likelihood ratios for jaw claudication, weight loss and PMR when compared to TAB studies. Absence of an elevated CRP provided a lower negative likelihood ratio in studies using the clinical diagnosis.Conclusion:Few clinical findings may help to estimate the clinical probability of GCA. The presence or absence of any particular symptom or sign does not sufficiently rule out or rule in GCA. These findings highlight the need of additional tests (i.e. imaging, biopsy) in patients suspected of having GCA.

Anatomoclinical results of temporal artery biopsies at a university hospital in Argentina

Giant cell arteritis (GCA) is the most frequent systemic vasculitis in patients older than 50 years. The diagnosis is based on the clinical history, laboratory findings and imaging studies associated with a temporal artery biopsy (TAB). However, the biopsy result could be inconclusive in up to 40% of the cases. The aim of this study was to review the current management of the patients with clinical suspect of GCA in the university hospital CEMIC in Buenos Aires, Argentina, and correlate the disease behavior with the TAB result. Retrospective study that reviewed consecutive patients to whom a TAB was made in an 11-year period (2005-2016). Clinical and pathology reports were reviewed. Descriptive statistics were performed. Quantitative variables were described as mean (S.D.) or median [range or inter- quartile range (IQR)] and qualitative variables as number (%). To compare the characteristics of the groups, bivariate analyzes were performed using contingency tables and logistic regression models if necessary. Sixty three patients were included, 68% women. The mean age was 72 years old (SD 8.4). Seventeen biopsies (26.9%) were positive for GCA. The average post fixation length was 1.68 cm (SD 1.2). Patients were divided into 3 groups taking into account the result of the TAB, the ACR criteria and the imaging studies. We could not identify predictors of biopsy positivity. The group of patients with GCA and negative TAB showed a higher percentage of patients with abnormal temporal artery at physical examination. The TAB positive percentage (26.9%) was similar to the reported in other series as well as the post fixation length. We could not identify predictors of biopsy positivity.

Neural network and logistic regression diagnostic prediction models for giant cell arteritis: development and validation.

PurposeTo develop and validate neural network (NN) vs logistic regression (LR) diagnostic prediction models in patients with suspected giant cell arteritis (GCA). Design: Multicenter retrospective chart review.MethodsAn audit of consecutive patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at 14 international medical centers. The outcome variable was biopsy-proven GCA. The predictor variables were age, gender, headache, clinical temporal artery abnormality, jaw claudication, vision loss, diplopia, erythrocyte sedimentation rate, C-reactive protein, and platelet level. The data were divided into three groups to train, validate, and test the models. The NN model with the lowest false-negative rate was chosen. Internal and external validations were performed.ResultsOf 1,833 patients who underwent TABx, there was complete information on 1,201 patients, 300 (25%) of whom had a positive TABx. On multivariable LR age, platelets, jaw claudication, vision loss, log C-reactive protein, log erythrocyte sedimentation rate, headache, and clinical temporal artery abnormality were statistically significant predictors of a positive TABx (P≤0.05). The area under the receiver operating characteristic curve/Hosmer–Lemeshow P for LR was 0.867 (95% CI, 0.794, 0.917)/0.119 vs NN 0.860 (95% CI, 0.786, 0.911)/0.805, with no statistically significant difference of the area under the curves (P=0.316). The misclassification rate/false-negative rate of LR was 20.6%/47.5% vs 18.1%/30.5% for NN. Missing data analysis did not change the results.ConclusionStatistical models can aid in the triage of patients with suspected GCA. Misclassification remains a concern, but cutoff values for 95% and 99% sensitivities are provided (https://goo.gl/THCnuU).

Diagnostic Utility of Computed Tomographic Angiography in Giant-Cell Arteritis.

Background and Purpose- The diagnosis of giant-cell arteritis (GCA) is challenging. Superficial temporal artery biopsy and ultrasound are positive in only 50%. We evaluated computed tomographic angiography (CTA) of the head in GCA. Methods- This case-control study was performed using a prospective GCA registry. Cases presented with stroke symptoms, had a CTA, and were subsequently diagnosed with GCA. Age- and sex-matched controls presented with stroke symptoms, had a CTA, and were not diagnosed with GCA. CTAs were evaluated for the presence of superficial temporal artery abnormalities. Results- Fourteen cases met the inclusion criteria and were matched with 14 controls. Blurred vessel wall margins and perivascular enhancement was found in 10 cases (71.4%) and 2 controls (14.3%). CTA has an accuracy of 78.6%, sensitivity of 71.4%, and a specificity of 85.7% for GCA. Conclusions- CTA detects superficial temporal artery abnormalities in GCA. This may facilitate early diagnosis and prompt implementation of potentially sight-saving and stroke-preventing treatment.

The Use of a Nomogram to Visually Interpret a Logistic Regression Prediction Model for Giant Cell Arteritis

ABSTRACTObjective: To illustrate the utility of a nomogram for the prediction of giant cell arteritis (GCA).Method: A nomogram was constructed from a multivariable logistic regression prediction model with 10 covariates: age, sex, clinical temporal artery abnormality, new-onset headache, jaw claudication, vision loss, diplopia, erythrocyte sedimentation rate, C-reactive protein, and platelet level.Results: The magnitude and location of the nomogram scale for each predictor variable graphically illustrates the net effect of each covariate and is especially useful for continuous variables such as age and bloodwork values.Conclusions: Nomograms allow integration and synthesis of the relative importance of clinical variables and provide a graphic representation of the odds ratios, p values, and confidence intervals of logistic regression prediction models. Although nomograms and prediction rules cannot substitute for clinical judgment, they help objectify and optimize the individualized risk assessments for patients with suspected GCA.

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.

Which aspects of a patient's history and physical are most useful in diagnosing giant cell arteritis (GCA)/temporal arteritis (TA)?

EVIDENCE-BASED ANSWER Jaw claudication, diplopia, and temporal artery abnormalities such as tenderness, thickening, beading, and decreased/absent pulse appear to have the highest specificity (92%-96%) for temporal artery biopsy (TAB)-proven GCA/TA. However, the absence of any one common symptom does not rule out GCA/TA (SOR: A, systematic review of cross-sectional studies).

Clinical predictors of positive temporal artery biopsy

ObjectiveWe investigated the ability of known clinical signs and symptoms, as well as common laboratory tests, to correctly predict a positive temporal artery biopsy. DesignA prospective cohort study. ParticipantsConsecutive patients in a tertiary referral centre undergoing temporal artery biopsy. MethodsClinical information was collected using a predesigned questionnaire. Pathology results and laboratory information were collected from digital patient records. Main Outcome measureThe predictive value of clinical signs, symptoms, and laboratory values of a positive temporal artery biopsy. ResultsOver a 3-year period, 259 patients were enrolled and 251 patients were analyzed. Sixty-one patients had a positive biopsy. Clinical features most predictive of a positive biopsy were jaw claudication (positive likelihood ratio [LR+] 2.31) and abnormal temporal artery pulse (LR+ 2.62). Receiver operating characteristic curves generated for erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelets values showed an area under curve (AUC) value of 0.71, 0.75, and 0.76, respectively. The initiation of steroids decreased the diagnostic utility of the ESR, CRP, and platelets values (AUC = 0.58, 0.61, and 0.63, respectively). ConclusionsA variety of clinical signs and symptoms were observed in patients referred for a temporal artery biopsy. Clinical signs and symptoms were less accurate in predicting a positive biopsy than laboratory tests. No combination of clinical signs and symptoms tested was able to predict giant cell arteritis with the certainty necessary to justify or withhold long-term steroid therapy.

Visual Complications in Patients with Biopsy-proven Giant Cell Arteritis: A Population-based Study.

To study the clinical and laboratory characteristics of patients with biopsy-proven giant cell arteritis (GCA) with visual complications, and to evaluate the incidence rate of visual complications in GCA compared to the background population. Data from 840 patients with GCA in the county of Skåne, Sweden, diagnosed between 1997 and 2010, were used for this analysis. Cases with visual complications were identified from a diagnosis registry and confirmed by a review of medical records. The rate of visual complications in patients with GCA was compared with an age- and sex-matched reference population. There were 85 patients (10%) who developed ≥ 1 visual complication after the onset of GCA. Of the patients, 18 (21%) developed unilateral or bilateral complete visual loss. The mean age at diagnosis was 78 years (± 7.3); 69% were women. Compared with patients without visual complications, those with visual complication had lower C-reactive protein levels at diagnosis and were less likely to have headache, fever, and palpable abnormal temporal artery. The use of β-adrenergic inhibitors was associated with visual complications. The incidence of visual complications among patients with GCA was 20.9/1000 person-years of followup compared to 6.9/1000 person-years in the reference population, resulting in a rate ratio of 3.0 (95% CI 2.3-3.8). Ten percent of patients with GCA developed visual complications, a rate substantially higher than that of the general population. Patients with GCA who had visual complications had lower inflammatory responses and were more likely to have been treated with β-adrenergic inhibitors compared with patients without visual complications.

Giant Cell Arteritis: Read the Fine Print!

Giant Cell Arteritis: Read the Fine Print!