Giant Cell Arteritis Patients Research Articles

Glucose metabolism controls disease-specific signatures of macrophage effector functions.

In inflammatory blood vessel diseases, macrophages represent a key component of the vascular infiltrates and are responsible for tissue injury and wall remodeling. To examine whether inflammatory macrophages in the vessel wall display a single distinctive effector program, we compared functional profiles in patients with either coronary artery disease (CAD) or giant cell arteritis (GCA). Unexpectedly, monocyte-derived macrophages from the 2 patient cohorts displayed disease-specific signatures and differed fundamentally in metabolic fitness. Macrophages from CAD patients were high producers for T cell chemoattractants (CXCL9, CXCL10), the cytokines IL-1β and IL-6, and the immunoinhibitory ligand PD-L1. In contrast, macrophages from GCA patients upregulated production of T cell chemoattractants (CXCL9, CXCL10) but not IL-1β and IL-6, and were distinctly low for PD-L1 expression. Notably, disease-specific effector profiles were already identifiable in circulating monocytes. The chemokinehicytokinehiPD-L1hi signature in CAD macrophages was sustained by excess uptake and breakdown of glucose, placing metabolic control upstream of inflammatory function. We conclude that monocytes and macrophages contribute to vascular inflammation in a disease-specific and discernible pattern, have choices to commit to different functional trajectories, are dependent on glucose availability in their immediate microenvironment, and possess memory in their lineage commitment. Supported by the NIH (R01 AR042527, R01 HL117913, R01 AI108906, P01 HL129941, R01 AI108891, R01 AG045779 U19 AI057266, R01 AI129191), I01 BX001669, and the Cahill Discovery Fund.

Giant cell arteritis in patients of Indian Subcontinental descent in the UK

GCA in the Indian Subcontinent (ISC) is rare. Our centre in London, UK, serves an ethnically diverse population, including a significant population of patients of ISC descent. We hypothesise that patients of ISC descent are no less likely than others to present with symptoms suggestive of GCA and therefore to undergo temporal artery biopsy (TAB). A retrospective audit of all TABs performed at our institution over an 8 year period, to identify ethnicity (white, black, ISC, other, unknown) and biopsy result. We compared the proportion of all patients of ISC descent attending the ED to the proportion of ISC patients undergoing TAB. We compared the proportion of positive TABs among ISC patients with positive TABs among white patients. We also compared the proportion of TAB in ISC patients with all non-ISC ethnicities combined. The proportion of patients undergoing TAB who were of ISC descent (16.3% of 92) was comparable to the proportion of A&E attendances made up by ISC patients [p = 0.1339]. 3.8% (1/26) of positive biopsies were among patients of ISC descent. White patients were significantly more likely to have a positive biopsy than patients of ISC ethnicity (33% of 61 white patients vs. 7% of 15 ISC [p = 0.0456]), as were patients of non-ISC ethnicity (32.5% of 77 non-ISC patients vs. 7% of 15 ISC patients [p = 0.0464]). At our centre, biopsy proven GCA occurs in patients of ISC descent, but rarely. Full investigation for GCA continues to be appropriate where it is suspected, regardless of ethnicity.

Giant-cell arteritis-related mortality in France: A multiple-cause-of-death analysis.

Giant-cell arteritis (GCA) is a large vessel vasculitis. Data regarding mortality are controversial. We describe the mortality data of the French death certificates for the period of 2005 to 2014. Using multiple-cause-of-death (MCOD) analysis, we calculated age-adjusted mortality rates for GCA, examined differences in mortality rates according to age and gender and analyzed the underlying causes of death (UCD). We analyzed 4628 death certificates listing a diagnosis of GCA as UCD or non-underlying cause of death (NUCD). The mean age of death was 86 (±6.8) years. The overall age-standardized mortality rate among GCA patients was 7.2 per million population. Throughout the study period, the mean age of death was significantly increased (r = 0.17, p < .0001) in both genders. There was no significant difference with age repartition of death in the general population (p = .26). When GCA was listed as the UCD, most frequent associated diseases were cardiovascular (79%) and infectious diseases (35%). When GCA was reported as the NUCD, the listed UCD was a cardiovascular event in 40% of cases, neoplasm in 13%, neurodegenerative disorder in 11% and infectious disease in 10%. When GCA was the UCD or NUCD, an age-adjusted observed/expected ratio > 1 in GCA-associated mortality compared with the general population mortality was observed for tuberculosis, pneumonia and cardiovascular diseases. In this analysis of French death certificates mentioning GCA, we observed a stable standardized mortality rate between 2005 and 2014. The most frequent associated diseases were cardiovascular diseases and infections.

PSY44 - TOCILIZUMAB IN COMBINATION WITH CORTICOSTEROIDS FOR THE TREATMENT OF GIANT CELL ARTERITIS: A BUDGET IMPACT ANALYSIS IN ITALY

To estimate the economic impact of tocilizumab (TCZ) administered weekly in combination with a 26-week prednisone taper regimen, for the treatment of giant cell arteritis (GCA), in the Italian market. A deterministic semi-Markov model was developed to simulate the evolution of GCA patients in Italy for 3 years. Baseline characteristics, transition probabilities and event rates were modelled according to the results of the phase III trial GiACTA. A comprehensive third-party payer perspective encompassing only direct health costs (drugs, resources consumption, and adverse events management) was adopted to evaluate the switch of all patients, currently treated with prednisone alone, to the new combination regimen of TCZ + prednisone. Target patients were estimated for 2019-2021 starting from Italian population (>50 years) applying national epidemiologic data and considering only patients eligible to receive TCZ. In the next three years 1,314 patients are expected to be treated: 161 in the first, 492 in the second and 661 in the third year of analysis. The switch to TCZ combination therapy results in an increase in the total cost of less than 15 million Euro for 3 years corresponding to 11,250 Euro/patient yearly. If the therapy adherence is included, the economic impact reduces to less than 12 million Euro for 3 years, corresponding to 8,775 Euro/patient yearly. Treatment cost of GCA depends almost completely on pharmacological therapy. Changes in the current prescription scenario, with the adoption of TCZ, have a sustainable economic impact; in particular, considering a realistic adherence.

Differences in clinical manifestations and prognosis of Chinese giant cell arteritis patients with or without polymyalgia rheumatica

BackgroundThe symptoms of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) frequently overlap in the elderly. Whether there are differences in clinical features or prognosis between GCA patients with or without PMR remains unknown.AimsTo identify differences in clinical manifestation and prognosis between Chinese GCA patients with or without PMR.MethodsA retrospective study of patients diagnosed with GCA in Peking Union Medical College Hospital (PUMCH) during the last 20 years was conducted. Clinical data was collected and analyzed accordingly, and follow-up was performed.ResultsA total of 50 patients had PMR, while 41 patients did not, with no significant differences in age, gender, and disease course between the two groups. GCA patients with PMR presented with higher risks of family history of malignancy (p = 0.048). Patients without PMR had higher proportion of hearing loss (p = 0.006), ANCA positive (p = 0.024), and abnormal imaging findings illustrating the involvement of arteries under aortic arch (p = 0.018). Before treatment, total lymphocyte counts in patients without PMR were lower than those with PMR, and monocyte counts in both groups were higher than normal. Acute phase reactants in patients without PMR were higher than the other group. No significant differences were found in prognosis during follow-up.ConclusionsGCA patients with or without PMR have different clinical characteristics. Patients with PMR present myalgia or arthralgia more frequently, while those without PMR have higher inflammatory markers, lower lymphocyte counts, and wider involvement of arteries under aortic arch.

Diagnosis and differential diagnosis of large-vessel vasculitides.

There are no universally accepted diagnostic criteria for large-vessel vasculitides (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK). Currently, available classification criteria cannot be used for the diagnosis of GCA and TAK. Early diagnosis of these two diseases is quite challenging in clinical practice and may be accomplished only by combining the patient symptoms, physical examination findings, blood test results, imaging findings, and biopsy results, if available. Awareness of red flags which lead the clinician to investigate TAK in a young patient with persistent systemic inflammation is helpful for the early diagnosis. It should be noted that clinical presentation may be highly variable in a subgroup of GCA patients with predominant large-vessel involvement (LVI) and without prominent cranial symptoms. Imaging modalities are especially helpful for the diagnosis of this subgroup. Differential diagnosis between older patients with TAK and this subgroup of GCA patients presenting with LVI may be difficult. Various pathologies may mimic LVV either by causing systemic inflammation and constitutional symptoms, or by causing lumen narrowing with or without aneurysm formation in the aorta and its branches. Differential diagnosis of aortitis is crucial. Infectious aortitis including mycotic aneurysms due to septicemia or endocarditis, as well as causes such as syphilis and mycobacterial infections should always be excluded. On the other hand, the presence of non-infectious aortitis is not unique for TAK and GCA. It should be noted that aortitis, other large-vessel involvement or both, may occasionally be seen in various other autoimmune pathologies including ANCA-positive vasculitides, Behçet's disease, ankylosing spondylitis, sarcoidosis, and Sjögren's syndrome. Besides, aortitis may be idiopathic and isolated. Atherosclerosis should always be considered in the differential diagnosis of LVV. Other pathologies which may mimic LVV include, but not limited to, congenital causes of aortic coarctation and middle aortic syndrome, immunoglobulin G4-related disease, and hereditary disorders of connective tissue such as Marfan syndrome and Ehler-Danlos syndrome.

Giant cell arteritis and inflammatory bowel disease - Is there a connection? Results from a population-based study.

Giant cell arteritis (GCA) is an autoimmune disorder which primarily affects large vessels, whilst inflammatory bowel diseases (IBD) mainly target the gut. Co-existence of the two maladies has been reported sporadically in the literature; therefore the purpose of this study was to assess the authenticity of such an association in a large, cross-sectional study. Utilizing data derived from the Clalit Health Services' registry, the largest health maintenance organization in Israel, we compared the proportion of CD and UC in GCA patients with age- and gender-matched controls. Univariate analysis was performed using Chi-square and student t-test and a multivariate analysis was performed using a logistic regression model. The study included 3938 GCA patients and 21,623 age- and gender-matched controls. GCA patients had a significantly increased proportion of both CD and UC in comparison with controls (0.79% vs. 0.12% and 0.84% vs. 0.21%, P-value < .001, respectively). The strength of the association between GCA and IBD was negatively correlated with the patients' age; thus the association was more robust amongst middle-aged patients (ages 50-69, OR = 8.13) than in elderly patients (ages 70-85, OR = 3.81). The association between GCA and IBD remained significant when evaluated independently of confounding factors (OR = 2.63, P-value < .001). The probability that GCA patients also suffer from IBD is increased in comparison with age- and gender-matched controls. Our findings indicate that this association is more prominent in middle-aged patients (50-69 years of age). Screening for IBD amongst GCA patients in this age group may be warranted.

Utility of serological biomarkers for giant cell arteritis in a large cohort of treatment-na\xefve patients

Early diagnosis and treatment of giant cell arteritis (GCA) is crucial for preventing ischemic complications. Multiple serological markers have been identified; however, there is a distinct lack of predicting markers for GCA relapse and complications. Our main objective was to identify serological parameters in a large cohort of treatment-naïve GCA patients, which could support clinicians in evaluating the course of the disease. Clinical data was gathered, along with analyte detection using Luminex technology, ELISA, and nephelometry, among others. Unsupervised hierarchical clustering and principal component analysis of analyte profiles were performed to determine delineation of GCA patients and healthy blood donors (HBDs). Highest, significantly elevated analytes in GCA patients were SAA (83-fold > HBDs median values), IL-23 (58-fold), and IL-6 (11-fold). Importantly, we show for the first time significantly changed levels of MARCO, alpha-fetoprotein, protein C, resistin, TNC, TNF RI, M-CSF, IL-18, and IL-31 in GCA versus HBDs. Changes in levels of SAA, CRP, haptoglobin, ESR, MMP-1 and MMP-2, and TNF-alpha were found associated with relapse and visual disturbances. aCL IgG was associated with limb artery involvement, even following adjustment for multiple testing. Principal component analysis revealed clear delineation between HBDs and GCA patients. Our study reveals biomarker clusters in a large cohort of patients with GCA and emphasizes the importance of using groups of serological biomarkers, such as acute phase proteins, MMPs, and cytokines (e.g. TNF-alpha) that could provide crucial insight into GCA complications and progression, leading to a more personalized disease management.

The clinical benefit of imaging in the diagnosis and treatment of giant cell arteritis.

Historically, giant cell arteritis (GCA) was considered to be synonymous with temporal arteritis. However, the disease spectrum of GCA extends much further, and includes vasculitis of the aorta and its branches with or without involvement of the temporal arteries. Imaging is crucial for the diagnosis and follow-up of GCA patients. Large vessel GCA (LV-GCA) often presents as an inflammatory syndrome and is only detected by imaging modalities such as: colour duplex sonography (CDS), computed tomography (CT) / CT angiography (CTA), magnetic resonance imaging (MRI) or 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) / CT. Deciding which imaging modality to use in different clinical situations remains a matter of debate. CDS and MRI enable assessment of the temporal arteries with a presumably higher sensitivity than histology. In the context of a typical presentation, CDS can replace a biopsy. In about a third of patients, the temporal arteries are not involved, thus PET/CT, MRI, CT, or CDS of larger arteries is needed to diagnose GCA. The sensitivity of all modalities is affected by glucocorticoid therapy. Therefore, without delaying therapy, imaging should be performed within a few days of treatment initiation. The use of PET/CT for the work-up of inflammatory syndromes in the elderly reveals vasculitis in approximately 20% of examined patients and uncover relevant diagnoses in the majority of remaining patients. The aorta should be routinely assessed in all GCA patients at diagnosis and during follow-up. MRA or CTA are best suited to characterise structural damage of larger arteries. The role of imaging in monitoring GCA disease activity still needs to be further defined.

Does leflunomide have a role in giant cell arteritis? An open-label study

Glucocorticoid monotherapy has been the mainstay treatment of giant cell arteritis (GCA) for decades. We aimed to evaluate the role of leflunomide as a steroid-sparing agent in GCA. This open-label study included incipient GCA patients followed for at least 48 weeks at a single secondary/tertiary rheumatology centre. At the time of diagnosis, patients received glucocorticoids. At week 12 of follow-up, leflunomide 10 mg qd was recommended as an adjunctive therapy to all patients without contraindications. The decision to start the leflunomide was patient-dependent. The number of relapses, a cumulative glucocorticoid dose during follow-up and treatment-related adverse events (AE) were recorded and compared between glucocorticoid-only and leflunomide groups. Seventy-six patients (65.8% female, median (IQR) age 73.7 (66.1–78.8) years) were followed for a median (IQR) 96 (86–96) weeks. Thirty out of 76 (39.5%) patients received leflunomide at week 12 (leflunomide group); the others continued treatment with glucocorticoid (glucocorticoid-only group). During the first 48 weeks of follow-up, 22 patients relapsed, 4 (13.3%) in leflunomide group and 18 (39.1%) in glucocorticoid-only group. The difference was statistically significant (p = 0.02; NNT 3.9 (95% CI 2.2–17.4)). Furthermore, 17/30 (56.7%) patients in the leflunomide group managed to stop glucocorticoid at week 48 (with one relapse (5.9%) shortly afterwards). The cumulative glucocorticoid dose at the last visit was lower in the leflunomide group than in the glucocorticoid-only group (p = 0.01). Our findings indicate the steroid-sparing effect of leflunomide in GCA.

Gene and miRNA expression in giant cell arteritis-a concise systematic review of significantly modified studies.

Giant cell arteritis (GCA) is a systemic vasculitis in individuals older than 50years, characterized by headaches, visual disturbances, painful scalp, jaw claudication, impairment of limb arteries, and systemic inflammation, among other symptoms. GCA diagnosis is confirmed by a positive temporal artery biopsy (TAB) or by imaging modalities. A prominent acute phase response with inflammation is the hallmark of the disease, predominantly targeting large- and medium-sized arteries leading to stenosis or occlusion of arterial lumen. To date, there are no reliable tissue markers specific for GCA. Scarce reports have indicated the importance of epigenetics in GCA. The current systematic review reports significantly changed candidate biomarkers in TABs of GCA patients compared to non-GCA patients using qPCR.

Simple dichotomous assessment of cranial artery inflammation by conventional 18F-FDG PET/CT shows high accuracy for the diagnosis of giant cell arteritis: a case-control study.

To estimate the diagnostic accuracy of conventional 18F-FDG PET/CT of cranial arteries in the diagnosis of giant cell arteritis (GCA). The study was a retrospective case-control study. The reference diagnosis was fulfillment of the 1990 ACR criteria for GCA. All patients had new-onset GCA. Conventional 18F-FDG PET/CT was performed before glucocorticoid treatment. Controls were age- and sex-matched patients with a previous history of malignant melanoma (MM) undergoing surveillance PET/CT >6months after MM resection. PET images were evenly cropped to include only head and neck and were assessed in random order by four nuclear medicine physicians blinded to reference diagnosis. Temporal (TA), maxillary (MA) and vertebral (VA) arteries were visually rated for 18F-FDG uptake. Interreader agreement was evaluated by Fleiss kappa. A total of 44 patients and 44 controls were identified. In both groups, the mean age was 69years (p = 0.45) and 25/44 were women. 35/41 GCA patientswere temporal artery biopsy positive (TAB). Considering only FDG uptake in TA and/or MA, diagnostic sensitivity and specificity was 64 and 100%. Including VA, sensitivity increased to 82% and specificity remained 100%. Interreader agreement was 91% and Fleiss kappa 0.82 for the PET diagnosis based on the cranial arteries. Conventional 18F-FDG PET/CT is an accurate and reliable tool to diagnose cranial arteritis in glucocorticoid-naïve GCA patients. The high diagnostic specificity suggests that TAB can be omitted in patients with 18F-FDG uptake in cranial arteries. 18F-FDG PET/CT performed in patients with suspected vasculitis should always include the head and neck.

Current patient care of giant cell arteritis in Rhineland-Palatinate

Giant cell arteritis (GCA) is one of the most common forms of inflammatory vasculitis in older patients. Because of possible irreversible vision deterioration, afastest possible diagnosis and therapy is of absolute importance. To date, there are still no reliable data to obtain an initial assessment of the outpatient health care situation of patients diagnosed with GCA in Rhineland-Palatinate. The specialists (neurologists, rheumatologists, ophthalmologists and general practitioners) participating in the statewide rheumatology network ADAPTHERA were questioned with the help of aquestionnaire regarding disease frequency, activity, drug therapy and possible comorbidities. In addition, the collected data were compared and supplemented by the ambulatory coding of the Association of Statutory Health Insurance Physicians in Rhineland-Palatinate. Based on the information provided by general practitioners, 272 GCA patients were treated in Rhineland-Palatinate during the survey period. The average duration of the disease until diagnosis was 3.6(SD ± 4.8) months. Drug therapy in the form of glucocorticoids was in first place followed by methotrexate, acetylsalicylic acid (ASA) and azathioprine. Cardiovascular diseases, chronic pain syndromes, depression, osteoporosis and diabetes mellitus were also described as comorbidities. The majority of patients with GCA are being cared for by general practitioners (GP). Long-term therapy and timely rheumatological co-treatment seem to be problematic. The primary care providers expressed their wishes for rheumatological training and further education measures. In terms of diagnosis and treatment, there is ademand to implement a"vasculitis fast-track" module.

Pericarditis among giant cell arteritis patients: From myth to reality.

Giant cell arteritis (GCA) is an inflammatory disease of unknown etiology affecting adults age > 50 years. GCA (also known as temporal arteritis) is a vasculitis of large and medium-size vessels that involves the extracranial branches of the carotid artery. Common manifestations include constitutional symptoms, headache, jaw claudication, scalp tenderness, and vision loss. Cardiac involvement in GCA is considered to be as low as 5%, and < 30 cases of pericarditis among GCA patients have been reported in the literature. The aim of this study was to evaluate the association between GCA and pericarditis by conducting a cross-sectional study utilizing the database of the largest healthcare provider in Israel. GCA is associated with pericarditis. The proportion of past documentation of pericarditis among patients diagnosed with GCA was compared with that of their age- and sex-matched controls. Univariate analysis was performed using the χ2 and t tests; multivariate analysis was performed using logistic regression. The study included 4329 GCA patients and 21 611 controls. GCA patients had higher rates of cardiovascular risk factors. Pericarditis was observed in 53 GCA patients and 72 controls (1.22% vs 0.33%, respectively; P < 0.001), significantly higher among GCA patients in comparison with controls. A significant interaction was found between GCA, pericarditis, and young age (<70 years). The study showed an independent association between GCA and pericarditis, especially among young patients. Proper screening should be applied whenever a suspicion arises as to the existence of comorbidity in patients with either disease.

Incremental Costs in Giant Cell Arteritis.

To assess and compare direct costs between giant cell arteritis (GCA) patients and matched controls and to identify incremental cost drivers. We carried out a population-based, retrospective cohort study using the French National Health Insurance System database. Cost analysis was performed from the French health insurance perspective and took into account direct medical and nonmedical costs (2014, €). Costs were evaluated according to different cost components and divided into periods of 6 months for the accurate assessment of care consumption. Longitudinal multivariate regression analyses using generalized estimating equations were used to adjust the effect of GCA on the mean cost over time. Analyses were performed on 96 incident GCA patients and 563 matched controls. The cumulative incremental cost due to GCA was €6,406 and €7,236 for 3 and 5 years, respectively. Total incremental costs were significant for the first 18 months, amounting to €1,342 for the first 6 months, €1,498 between 6 and 12 months, and €1,165 between 12 and 18 months (P = 0.012, P = 0.065, and P = 0.029, respectively). The most important cost drivers were paramedical procedures, inpatient stays, medication, and medical procedures. Multivariate analysis shows the significant effect of GCA on mean cost during the first 3 years of followup (relative risk [RR] 1.72 [95% confidence interval (95% CI) 1.31-2.27], P < 0.001) with significant cost reductions (RR 0.70 [95% CI 0.49-0.99], P = 0.05) at the end of followup. This study provides an accurate assessment of GCA costs during a 5-year period and gives useful information for future cost-effectiveness studies based on new expensive biotherapies.

Expression and Function of IL12/23 Related Cytokine Subunits (p35, p40, and p19) in Giant-Cell Arteritis Lesions: Contribution of p40 to Th1- and Th17-Mediated Inflammatory Pathways.

Giant-cell arteritis (GCA) is considered a T helper (Th)1- and Th17-mediated disease. Interleukin (IL)-12 is a heterodimeric cytokine (p35/p40) involved in Th1 differentiation. When combining with p19 subunit, p40 compose IL-23, a powerful pro-inflammatory cytokine that maintains Th17 response. The aims of this study were to investigate p40, p35, and p19 subunit expression in GCA lesions and their combinations to conform different cytokines, to assess the effect of glucocorticoid treatment on subunit expression, and to explore functional roles of p40 by culturing temporal artery sections with a neutralizing anti-human IL-12/IL-23p40 antibody. p40 and p19 mRNA concentrations measured by real-time RT-PCR were significantly higher in temporal arteries from 50 patients compared to 20 controls (4.35 ± 4.06 vs 0.51 ± 0.75; p < 0.0001 and 20.32 ± 21.78 vs 4.17 ± 4.43 relative units; p < 0.0001, respectively). No differences were found in constitutively expressed p35 mRNA. Contrarily, p40 and p19 mRNAs were decreased in temporal arteries from 16 treated GCA patients vs those from 34 treatment-naïve GCA patients. Accordingly, dexamethasone reduced p40 and p19 expression in cultured arteries. Subunit associations to conform IL-12 and IL-23 were confirmed by proximity-ligation assay in GCA lesions. Immunofluorescence revealed widespread p19 and p35 expression by inflammatory cells, independent from p40. Blocking IL-12/IL-23p40 tended to reduce IFNγ and IL-17 mRNA production by cultured GCA arteries and tended to increase Th17 inducers IL-1β and IL-6. IL-12 and IL-23 heterodimers are increased in GCA lesions and decrease with glucocorticoid treatment. p19 and p35 subunits are much more abundant than p40, indicating an independent role for these subunits or their potential association with alternative subunits. The modest effect of IL-12/IL-23p40 neutralization may indicate compensation by redundant cytokines or cytokines resulting from alternative combinations.

CT analysis of the aorta in giant-cell arteritis: a case-control study

Giant cell arteritis (GCA) is a large-vessel vasculitis whose diagnosis is confirmed by temporal artery biopsy. However, involvement of large vessels, especially the aorta, can be shown by imaging, which plays an increasing role in GCA diagnosis. The threshold above which aortic wall thickening, as measured by computed tomography (CT), is considered pathological is controversial, with values ranging from 2 to 3 mm. This study assessed aortic morphology by CT scan and its diagnostic value in GCA. Altogether, 174 patients were included (64 with GCA, 43 with polymyalgia rheumatica and 67 controls). All patients had a CT scan at diagnosis or at inclusion for controls. Aortic wall thickness, aortic diameter and scores for atheroma were measured. Assessor was blinded to each patient's group. Aortic diameters and atheroma scores were similar between groups. Aortic wall thickness was greater in the GCA group, even after the exclusion of GCA patients with aortic wall thickness ≥3 mm. The receiver operating characteristic (ROC) curve showed that a wall thickness of 2.2 mm was the optimal threshold to diagnose GCA (sensitivity, 67%; specificity, 98%). Measuring aortic wall thickness by CT scan is effective to diagnose GCA. The optimal threshold to regard aortic wall thickening as pathological was ≥2.2 mm. • Imaging, including CT scan, plays an increasing role in GCA diagnosis • CT measurement of aortic wall thickness is useful to diagnose GCA • A 2.2-mm threshold allows the diagnosis of thickened aortic wall in GCA.

Large-vessel involvement and aortic dilation in giant-cell arteritis. A multicenter study of 549 patients

Large-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA. A retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model. The patients with LVI were younger (p<0.0001), more likely to be women (p=0.01), and showed fewer cephalic symptoms (p<0.0001) and polymyalgia rheumatica (p=0.001) but more extracranial vascular symptoms (p=0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p=0.0003). Repeated aortic imaging procedures were performed at 19months [range: 5-162months] and 17months [range: 6-168months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p=0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34-7.48], p=0.009). LVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required.

Unexpected Visual Loss in Appropriately Treated Giant Cell Arteritis

Unexpected Visual Loss in Appropriately Treated Giant Cell Arteritis

Herpes Zoster as a Risk Factor for Incident Giant Cell Arteritis.

Histopathologic studies have implicated herpes zoster (HZ) as a causative organism of giant cell arteritis (GCA). The purpose of this study was to assess the epidemiologic association of HZ events with incident GCA. We performed a retrospective cohort study in 2 large independent US administrative data sets: Medicare 5% and Truven Health Analytics MarketScan. Eligible subjects had 12 months of continuous coverage, were >50 years old, and had no history of GCA or polymyalgia rheumatica. HZ events (complicated and uncomplicated) and GCA were identified by the presence of International Classification of Diseases, Ninth Revision, Clinical Modification codes from physician visit or hospital discharge records. Antiviral therapies and vaccinations were identified from prescription claims and drug codes. Risk of incident GCA was calculated using multivariable Cox proportional hazards regression. Among 16,686,345 subjects, a total of 5,942 GCA cases occurred, with 3.1% (MarketScan) and 6.0% (Medicare) having preceding HZ events. Unadjusted GCA incidence rates were highest in the groups with complicated and uncomplicated HZ. After multivariable adjustment, complicated HZ was associated with an increased risk of GCA (hazard ratio [HR] 1.99 [95% confidence interval (95% CI) 1.32-3.02] in the Medicare cohort and 2.16 [95% CI 1.46-3.18] in the MarketScan cohort), as was uncomplicated HZ (HR 1.42 [95% CI 1.02-1.99] and HR 1.45 [95% CI 1.05-2.01] in the respective cohorts). Vaccination and antiviral treatment were not consistently associated with GCA risk, although antiviral treatment was marginally associated with a decreased risk of GCA in the Medicare cohort (HR 0.67 [95% CI 0.46-0.99]). HZ is associated with an increased risk of GCA. The infrequency of HZ in GCA patients suggests that it is only one potential trigger for GCA. Antivirals and vaccination did not consistently mitigate this risk.