Classification Of Giant Cell Arteritis Research Articles

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.

Incidence of primary large vessel vasculitis in Norfolk, UK from 2011 to 2020

ObjectivesTo report the annual incidence of primary large vessel vasculitis (LVV) in the adult population of Norfolk County, UK, including giant cell arteritis (GCA) (in those ≥50 years) and Takayasu...

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.

Cranial and extracranial large-vessel giant cell arteritis share a genetic pattern of interferon-gamma pathway.

Two main different clinical phenotypes of giant cell arteritis (GCA) have been described, the classic cranial pattern and the extracranial large-vessel (LV) pattern. Since interferon gamma (IFNG) has shown to be a pivotal cytokine in the pathophysiology of GCA, our aim was to evaluate for the first time the influence of IFNG and IFNG receptor 1 (IFNGR1) polymorphisms in the different clinical phenotypes of GCA. Two IFNG polymorphisms (rs2069718 G/A and rs1861493 A/G) and one polymorphism in IFNGR1 (rs1327474 G/A) were genotyped in 191 patients with biopsy-proven cranial GCA, 109 with extracranial LV-GCA and 490 healthy controls. A comparative study was conducted between patients with cranial and extracranial LV-GCA. No significant differences in genotype, allele, and haplotype frequencies of IFNG polymorphisms were found between GCA patients with the classic cranial pattern and the extracranial LV-GCA pattern. Similar results were found for genotype and allele frequencies of IFNGR1 polymorphism. It was also the case when patients with extracranial LV-GCA were compared with healthy controls. Our results show that IFNG and IFNGR1 polymorphisms do not influence the clinical phenotype of expression of GCA. Classic cranial GCA and extracranial LV-GCA seem to share a genetic pattern of IFNG pathway.

Giant Cell Arteritis Presenting With Ocular Symptoms: Clinical Characteristics and Multimodal Imaging in a Chinese Case Series.

PurposeTo evaluate demographic and clinical characteristics of a Chinese population with giant cell arteritis using multimodal imaging focusing on ophthalmic examinations.DesignRetrospective observational case series.Materials and MethodsIn the neuro-ophthalmology division of the Eye, Ear, Nose, and Throat Hospital, Shanghai, we evaluated the demographic and clinical characteristics of patients diagnosed with giant cell arteritis between January 2016 and June 2021. Results of routine ophthalmic examinations including fundus examination, optical coherence tomography, color duplex ultrasonography of ocular and superficial temporal arteries, orbital magnetic resonance imaging, and superficial temporal artery biopsy were evaluated.ResultsA total of 15 patients (22 eyes; ten male and five female) were evaluated with a mean age of 77.0 ± 8.5 years. Among them, seven had bilateral involvement that occurred simultaneously or sequentially. Twelve patients presented with arteritic anterior ischemic optic neuropathy, two with arteritic anterior ischemic optic neuropathy combined with cilioretinal artery occlusion, and one with cotton-wool spots. In acute stages of optic neuropathy and retinopathy, optical coherence tomography revealed optic disc edema, thickening of the inner retinal nerve fiber layer and ganglion cell layer, and loss of layer structure. In late stages, optical coherence tomography revealed diffuse atrophy of the inner retina. The “halo” sign was observed in 12 patients in the superficial temporal artery ultrasound, and seven out of eight patients who underwent biopsy demonstrated classic giant cell arteritis pathological changes. Most patients having poor visual acuity but ability to perceive light; 10/22 eyes had permanent vision loss.ConclusionAlthough rare in Asians, giant cell arteritis may be underdiagnosed among elderly Chinese patients presenting with anterior ischemic optic neuropathy. Non-invasive superficial temporal artery ultrasound detecting inflammatory thickening of the intima as the “halo” sign combined with routine elevated erythrocyte sedimentation rate and C-reactive protein may be helpful in diagnosing patients with a high probability of having giant cell arteritis.

Giant Cell and Takayasu Arteritiss

Many diseases cause inflammatory large vessel vasculitis. However, giant cell arteritis (GCA) and Takayasu arteritis (TA) are the most common large vessel vasculitides. Vascular surgeons should be aware of the workup and management of these two large vessel vasculitides as they are often involved in the care of these patients. Medical management is the primary therapy for both GCA and TA. However, surgical or endovascular therapy may be necessary in certain circumstances. This review explores all of the above aspects of both GCA and TA. Tables highlight inflammatory large vessel vasculitides, major differences between GCA and TA, criteria for the classification of GCA and TA, the distribution of arterial lesions in GCA and TA, and clinical presentation in TA patients by symptom. Figures show occlusive or aneurysmal disease of the aorta and its branches; pallid and sectoral edema; external carotid artery, superficial temporal artery, and facial nerve anatomy; frequency of arterial involvement in GCA and TA; the placement of incision for superficial temporal artery biopsy; right subclavian and axillary arteries in TA; and histology of the superficial temporal artery and a superficial temporal artery in GCA. This review contains 12 figures, 6 tables, and 89 references.

Vascular endothelial growth factor haplotypes are associated with severe ischaemic complications in giant cell arteritis regardless of the disease phenotype.

To determine whether functional vascular endothelial growth factor (VEGF) polymorphisms influence the expression of the clinical phenotype of giant cell arteritis (GCA). We also evaluated whether VEGF polymorphism is associated with the development of severe ischaemic manifestations in patients with GCA regardless of the clinical phenotype, classic cranial GCA or predominantly extracranial GCA large vessel vasculitis (LVV). VEGF rs833061 T/C, rs2010963 G/C and rs3025039 C/T polymorphisms were genotyped in 185 patients with biopsy-proven cranial GCA, 105 with extracranial LVV-GCA and 490 healthy controls. Allelic combinations (haplotypes) of VEGF were carried out. Comparisons were performed between patients with GCA and healthy controls as well as between patients with GCA stratified according to the clinical phenotype and the presence of severe ischaemic manifestations. No significant differences in genotype, allele, and haplotype frequencies of VEGF were found between patients with GCA and healthy controls as well as between GCA patients with the classic cranial pattern and the extracranial LVV-GCA pattern of the disease. However, the VEGF CGC haplotype (OR= 1.63 [1.05-2.53]) and the CGT haplotype (OR= 2.55 [1.10-5.91]) were significantly more frequent in GCA patients with severe ischaemic complications compared to those patients without these complications. VEGF haplotypes seem to play a role in the development of severe ischaemic manifestations in GCA patients, regardless of the clinical phenotype of expression of the disease.

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.

The presence of both HLA-DRB1*04:01 and HLA-B*15:01 increases the susceptibility to cranial and extracranial giant cell arteritis.

To determine if patients with the predominant extracranial large-vessel-vasculitis (LVV) pattern of giant cell arteritis (GCA) have a distinctive HLA-B association, different from that reported in biopsy-proven cranial GCA patients. In a further step we assessed if the combination of HLA-B and HLA-DRB1 alleles confers an increased risk for GCA susceptibility, either for the cranial and extracranial LVV phenotypes. A total of 184 patients with biopsy-proven cranial GCA, 105 with LVV-GCA and 486 healthy controls were included in our study. We compared HLA-B phenotype frequencies between the three groups. HLA-B*15 phenotype was significantly increased in patients with classic cranial GCA compared to controls (14.7% versus 5.8%, respectively; p<0.01; OR [95% CI] =2.81 [1.54-5.11]). It was mainly due to the HLA-B*15:01 allele (12.5% versus 4.0%, respectively; p<0.01; OR [95% CI] =3.51 [1.77-6.99]) and remained statistically significant after Bonferroni correction. Similar HLA-B*15 association was observed in patients with the LVV-GCA (11.4% versus 5.8%, p=0.04, OR [95% CI] =2.11 [1.04-4.30]). This association was also mainly due to the HLA-B*15:01 allele (10.5% versus 4.0%, respectively; p=0.0054; OR [95% CI] =2.88 [1.19-6.59]). Noteworthy, the presence of HLA-B*15:01 together with HLA-DRB1*04:01 led to an increased risk of developing both cranial and extracranial LVV-GCA. Susceptibility to GCA is strongly related to the HLA region, regardless of the clinical phenotype of expression of the disease.

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.

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.

Cranial and extracranial giant cell arteritis share similar HLA-DRB1 association

ObjectiveTo determine whether giant cell arteritis (GCA) patients with the typical pattern of cranial ischemic manifestations and those with the extracranial large-vessel-vasculitis (LVV)-GCA phenotype exhibit different HLA-DRB1 association. Methods178 biopsy-proven GCA patients who had cranial ischemic features but no LVV manifestations, 100 patients with LVV-GCA without cranial ischemic manifestations and 486 ethnically matched healthy controls were recruited. All patients and controls were Spanish of European ancestry. We compared HLA-DRB1 phenotype frequencies between the three groups. ResultsBoth GCA subgroups had well-differentiated clinical features. Patients with LVV-GCA were younger (68.0 ± 10.0 years versus 74.0 ± 10.4 years; p < 0.01) and presented more commonly with polymyalgia rheumatica symptoms (81% versus 39.3%; p < 0.01) than those with the classic cranial GCA phenotype. HLA-DRB1*04 phenotype frequency was significantly increased in patients with classic cranial GCA compared to controls (42.1% versus 23.5%, respectively; p < 0.01; odds ratio-OR [95% confidence interval-CI] = 2.38 [1.62–3.47]). This association was mainly due to the HLA-DRB1*04:01 allele (20.8% versus 5.3%, respectively; p < 0.01; OR [95% CI] = 4.64 [2.63–8.26]). HLA-DRB1*04 association was also observed in LVV-GCA patients when compared to controls (46.0% versus 23.5%, respectively; p < 0.01; OR [95% CI] = 2.78 [1.73–4.44]). Similar to cranial GCA, the association was also mainly due to the HLA-DRB1*04:01 allele (19.0% versus 5.3%, respectively; p < 0.01; OR [95% CI] = 4.15 [2.06–8.19]). Cranial and LVV-GCA patients did not exhibit HLA-DRB1 allele differences. ConclusionCranial and extracranial LVV-GCA share similar HLA-DRB1 association.

P180 External validation of the Southend GCA Probability Score (GCAPS) as a screening tool for referrals with possible GCA

Abstract Background We run a rheumatologist-led, ultrasound-driven giant cell arteritis (GCA) fast track pathway (FTP). Currently, no specific referral criteria are required. Sensitive referral criteria are now needed to safely reduce referrals, which have risen year-on-year (92 in Year 3, 84% greater than Year 1). Methods The following were collected for all patients referred to our GCA-FTP from 1/4/2018-31/3/2019 (Year 3): Final diagnosis of imaging/biopsy confirmed-GCA, unconfirmed clinical-GCA or not-GCA using the electronic patient record to September 2019; Rheumatologist-determined clinical probability of GCA (low, moderate, high) before imaging/biopsy; Presence of ≥ 3 ACR 1990 GCA Classification Criteria (biopsy excluded, adapted to increase sensitivity, see table); Presence of Southend GCAPS≥10 (Based on our clinical experience, polymyalgic symptoms were also counted if patients had recently taken prednisolone for PMR). Results 88 consecutive patients were analysed (total 92: 2 missing data; 2 passed through twice). 25/88 (27%) were diagnosed with GCA 24 were imaging/biopsy confirmed. The single patient with unconfirmed clinical-GCA had high clinical probability, indeterminate ultrasound and biopsy after &amp;gt;1-week glucocorticoid. GCA patients were mean age 77.3y (range 63-94), 76% female, all White, with mean GCAPS 15.3 (range 10-25). Our adaptations of the ACR criteria increased ACR-criteria sensitivity: 24% GCA patients had new generalised headache and 16% had ESR&amp;lt;50, but CRP&amp;gt;10. 14% referrals were non-White, although GCA is rare in this group. GCAPS was based on a largely White population. If GCAPS did not remove 3 points for alternative diagnoses, then 5 additional non-White patients would have scored ≥10. Conclusion This study externally validates GCAPS≥10 as a screening tool for referrals with possible GCA. It captured all GCA patients and screened out 44% referrals. For this indication, it performed better than a consultant rheumatologist. Despite adaptations to improve sensitivity, ACR criteria missed a quarter of cases. To improve GCAPS specificity when scored by a non-rheumatologist, GCAPS should be adapted to reflect the low likelihood of GCA in non-Whites. We plan to only accept patients with GCAPS≥10, where 3 points are removed for non-Whites if not already removed for alternative diagnoses. We predict referrals will reduce but anticipate referrers will need education to limit over-scoring. Disclosures V. Quick None. M. Hughes None. N. Mothojakan None. D. Fishman None.

Role of positron emission tomography in the assessment of disease burden and risk of relapse in patients affected by giant cell arteritis.

Giant cell arteritis (GCA) is characterized by two subsets: cranial GCA and large-vessel GCA (LV-GCA); positron emission tomography (PET) is an essential tool in the diagnosis of LV-GCA. In this study, we aimed to investigate its potential prognostic value in the stratification of relapse risk. We retrospectively revised all the clinical records of patients who received a diagnosis of GCA at an immuno-rheumatology clinic of a University Hospital along 6 years and who underwent to a PET-CT examination at diagnosis. Clinical, laboratory, and imaging data were collected. Relapses were recorded. The study population included 19 patients (10 females, 52.6%; median age 74.0 [65.5-76.0] years), 12 with typical cranial GCA (63%) and 7 (37%) with LV-GCA. Based on PET findings, a diagnosis of aortitis was made in 15/19 patients, including 8/12 patients with a classical cranial GCA (58%). Along a median follow-up of 15 months [4.5-26.5], 4 relapses were observed. All relapsers were male; indeed, the difference in gender distribution was the only variable reaching statistical difference between relapsers and non-relapsers. Specifically, aortitis was not more frequent among relapsers. Our study confirms PET as a valid tool in the identification of LV-GCA with no cranial involvement. We failed to demonstrate a role for PET in the prognostic stratification of GCA, while male gender is suggested as a potential risk factor for GCA relapse.Key Points• A significant proportion of patients with GCA presents with non-cranial disease, the identification of which requires imaging studies, among which PET is particularly useful.• Aortitis might be detected also in patients with the classical, cranial GCA type but does not seem to have prognostic implications, at least in terms of relapse risk.• Male gender is suggested as a risk factor for relapse in GCA.

The Spectrum of Pericardial Involvement in Giant Cell Arteritis and Polymyalgia Rheumatica: A Systematic Review of Literature.

Giant cell arteritis (GCA) is a systemic vasculitis that commonly co-occurs with polymyalgia rheumatica (PMR) in elderly patients. Pericardial disease is an unusual manifestation of these inflammatory conditions, which has been reported only in case reports and small observational studies. However, no extensive research has been performed to study the demographics and clinical history of GCA or PMR patients with concomitant pericardial features. As a result, the medical evidence to help guide the physicians when evaluating such individuals is limited. To perform a systematic review of the medical literature in order to summarize the epidemiological and clinicopathological aspects of this unique association. We conducted an extensive search of PubMed, Cochrane Library, Ovid, Google Scholar, and gray literature to identify all the cases of GCA and PMR with pericardial involvement. The demographics, clinical features, and outcomes of the final cohort were reviewed and analyzed. The analysis comprised 52 clinical cases (51 identified from 46 articles and 1 from the residents' clinic). These included 44 patients with GCA and 8 with PMR. The mean age at presentation was 69.5 years, with only 46% of patients older than 70 years. The most common abnormality was pericardial effusion (85%), and in 37%, the pericardial event was the initial disease manifestation. Although a significant proportion of the patients were symptomatic (69%), the classic cranial symptoms were present in only 40%. Overall, the outcome was good even in the presence of large-vessel disease, which is usually a poor prognostic factor in classic GCA. On group analysis, patients with PMR were more likely to develop cardiac tamponade (37.5%; odds ratio, 25.8; confidence interval, 2.2-297.5; p = 0.01), whereas those with GCA were more likely to have large-vessel vasculitis (43%; odds ratio, 5.18; confidence interval, 0.58-252.1; p = 0.04). This study illustrates that patients with pericardial involvement represent a clinical phenotype of GCA (and possibly PMR), which is quite different from the cranial or large-vessel forms. These patients have a better prognosis likely due to younger age and presence of more overt symptoms resulting in early diagnosis.

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.

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...

FRI0472 Correlations between Different Histological Subsets of GIANT Cell Arteritis and Clinical Manifestations in A Large Monocentric Cohort of Biopsy Positive GCA Patients

BackgroundTemporal artery biopsy (TAB) showing transmural inflammation is considered the gold standard for the diagnosis of giant cell arteritis (GCA). In some cases of GCA, inflammation is confined to the...

The diagnosis and classification of giant cell arteritis

Giant-cell arteritis (GCA) involves the major branches of the aorta with predilection for the extracranial branches of the carotid artery. It occurs in individuals older than 50 years and the incidence increases with age. The signs and symptoms of giant cell arteritis can be classified into four subsets: cranial arteritis, extracranial arteritis, systemic symptoms and polymyalgia rheumatica. Patients may develop any combination of these manifestations, associated with laboratory evidence of an acute-phase reaction. The only test that confirms GCA diagnosis is a temporal artery biopsy, showing vasculitis with mononuclear cell inflammatory infiltrates, often with giant cells. Due to the focal and segmental nature of the infiltrates, areas of inflammation may be missed by the biopsy and the histological examination is normal in about 15% of the cases. Some imaging modalities may aid in the diagnosis of GCA. Among those, color duplex ultrasonography of the temporal arteries is more commonly used. There are no independent validating criteria to determine whether giant cell arteritis is present when a temporal artery biopsy is negative. The American College of Rheumatology criteria for the classification of giant cell arteritis may assist in the diagnosis. However, meeting classification criteria is not equivalent to making the diagnosis in individual patients, and the final diagnosis should be based on all clinical, laboratory, imaging and histological findings. Glucocorticoids are the treatment of choice for GCA. The initial dose is 40–60 mg/day for most uncomplicated cases. Addition of low-dose aspirin (100 mg/d) has been shown to significantly decrease the rate of vision loss and stroke during the course of the disease.