Patients with polymyalgia rheumatica (PMR) may have subclinical large vessel vasculitis. We compared the incidence of aortic complications in PMR and giant cell arteritis (GCA) to the general population.

A retrospective cohort study was performed of patients with PMR and GCA identified by ≥2 ICD-9/ICD-10-CM diagnostic codes and concurrent corticosteroid treatment in the US-based TriNetX database (2000-2024). Matched general population controls were identified (1:3 ratio). The primary outcome, aortic complications, was a composite of aortic aneurysm and dissection/rupture. Adjusted hazard ratios (aHR) were calculated using Cox proportional cause-specific hazard models with PMR as the referent category.

Of 57,336 patients, 17,327 had PMR, 4,734 had GCA, and 35,275 were matched controls. Median follow-up time was 3.74 years (interquartile range, 1.8-6.4). The incidence rate of any aortic complication per 1,000 person-years was highest for GCA (11.69), followed by PMR (6.78) and the general population (5.09). Compared to patients with PMR, patients with GCA had a higher risk of any aortic complication (aHR 1.87, 95 % confidence interval (CI) 1.58-2.21); the general population risk was similar (aHR 0.95, 95 % CI 0.84-1.06). In a sensitivity analysis, patients with PMR who later developed GCA had a risk similar to those initially diagnosed with GCA (aHR 0.85, 95 % CI 0.60-1.19).

Patients with PMR had a similar risk of large vessel complications compared to the general population and a lower risk compared to those with GCA. These results do not support screening for aortic inflammation among patients with PMR who lack features of GCA.

Tocilizumab (TCZ) is the only biologic approved in Giant Cell Arteritis (GCA). In clinical trials around a quarter of patients relapse during TCZ treatment. We assess the frequency, features and factors associated with relapses in a wide series of GCA patients in a real-world setting.

National multicenter observational study of GCA patients treated with TCZ between 2016 and 2021. The variables collected at TCZ initiation were demographic, clinical, laboratory, temporal artery biopsy, and imaging findings, corticosteroids dose, previous therapies and TCZ therapeutic schedule. We perform a comparative study between patients with/ without relapses (bivariate analysis) and a study of factors associated with relapse (multivariate logistic).

We study 407 patients (295 women; mean age 73.6 ± 8.9 years). After a mean follow-up of 25.3 ± 21.7 months, relapses were observed in 63 of 407 (15.5 %) patients. At TCZ initiation, no differences were observed between both groups (with/without relapses) in demographic, clinical and laboratory features or corticosteroid dose. The median time to the first relapse was 12 [6-24] months being the most frequent manifestations polymyalgia rheumatica (47.6 %), and headache (12.7 %). In multivariate logistic regression analysis, the set of variables associated with GCA relapses were TCZ initiation later than 6 weeks (OR 3.446 [1.196- 9.931]), optimization (OR 2.803 [1.507-5.215]) and administration of IV TCZ (OR 2.327 [1.244-4.353]) and previous therapies to TCZ (OR 5.062[2.402-10.665]).

In this series, GCA relapses were observed in 15 % of patients, all of them non-severe. Relapses were associated with TCZ therapeutic schedule, such as IV administration, optimization, delayed initiation and previous therapies to TCZ.

To determine clinical characteristics, outcome and occurrence of comorbidities in patients with biopsy-confirmed giant cell arteritis (GCA) treated with intravenous methylprednisolone (IVMP) vs those receiving oral glucocorticoids (OGC) only.

A retrospective study included patients with GCA diagnosed from 2004 through 2019. Clinical and laboratory characteristics, and cumulative GC dose were compared in patients receiving IVMP vs OGCs. Changes in visual acuity (VA), occurrence of comorbidities after GCA diagnosis, and mortality were analysed.

A total of 419 patients (69% female) were included. In total, 111 patients were initially treated with IVMP, 104 (94%) of whom showed visual manifestations at onset and 308 received OGCs only. Ninety patients (21.5%) exhibited visual involvement at onset, verified by an ophthalmologist. Compared with OGC, patients receiving IVMP exhibited lower inflammatory response at presentation. There was a tendency for improvement in VA with the use of IVMP, but the results were not statistically significant (OR 1.19, 95% CI 0.35-4.01). Patients treated with IVMP had a higher risk of newly diagnosed diabetes mellitus within a year of GCA diagnosis (OR 2.59, 95% CI 1.19-5.63). This risk remained elevated after adjusting for cumulative OGC dose at three months (adjusted OR 3.30, 95% CI 1.29-8.43). There was no difference in survival between treatment groups.

Our study found no evidence supporting any benefit of using IVMP in improving VA or survival. IVMP may increase diabetes risk within a year of GCA diagnosis. Further studies are needed to evaluate the value of IVMP in GCA.

Aortitis and periaortitis refer to the inflammation of the aortic wall and the surrounding tissues. Both conditions are associated with various diseases and express nonspecific manifestations. Early diagnosis and treatment are crucial to improve the prognosis of the disease. This study aimed to assess the causes and main clinical features of aortitis and periaortitis in patients from a single centre in Spain. Observational, retrospective study of patients diagnosed with aortitis or periaortitis at a Spanish referral center over the last decade. 134 patients (87 female; mean age of 55.1 ± 9.1 years) were recruited, 132 of which had aortitis and two periaortitis. Aortitis was associated with giant cell arteritis (n = 102), Takayasu's arteritis (n = 6), IgG4-related disease (n = 6), infectious diseases (n = 3), malignancy (n = 1), drugs (n = 1), isolated aortitis (n = 1), and other immune-mediated inflammatory diseases (IMIDs) (n = 12). IMIDs included were Sjögren's syndrome (n = 2), sarcoidosis (n = 2), rheumatoid arthritis (n = 2), axial spondyloarthritis (n = 2), inflammatory bowel disease (n = 1), primary biliary cirrhosis (n = 1), idiopathic pulmonary fibrosis (n = 1), and polyarteritis nodosa (n = 1). Periaortitis was due to idiopathic retroperitoneal fibrosis in both cases. Imaging techniques used for diagnosis included 18F-FDG PET/CT scan (n = 133), CT-angiography (n = 44), and/or MRI-angiography (n = 33). Polymyalgia rheumatica (52.2%) and asthenia (53.7%) were the most common manifestations, followed by limb claudication (23.9%) and inflammatory back pain (26.9%). Acute-phase reactants were typically increased. Aortitis is a common condition and may be associated with multiple non-infectious diseases. Its clinical presentation is often unspecific, requiring a high level of suspicion to get an early diagnosis and treatment.

This study aimed to assess the relative frequency of clinical features of giant cell arteritis (GCA) and to investigate the predictors of temporal artery biopsy (TAB) outcomes.

A literature search of Pubmed/Medline, Embase, ScienceDirect, Scopus, Web of Science (WoS), and Directory of Open Access journals (DOAJ) was conducted from January 1, 1990 to February 2025. Observational studies that reported original data on clinical features in patients diagnosed with GCA in accordance with 1990 ACR and/or 2022 ACR/EULAR classification criteria were deemed for inclusion. A random-effects meta-analysis was performed to determine the pooled prevalence estimates. The study's design adhered closely to the MOOSE standards. The JBI appraisal tool was used to evaluate the risk of bias. The study's protocol was pre-registered on PROSPERO (ID: CRD42024584763).

Out of initial 12,628 records, 62 articles (9971 patients) met all of the eligibility criteria. Mean patients' age upon diagnosis was 74.33 years (95%CI: 74.12-74.54 years). The most prevalent clinical feature of GCA was new-onset headache (75.7%; 95CI%: 72.2-79.0; 95%PI: 0.47-0.92). Other common symptoms of GCA were temporal artery abnormalities (51.5%; 95%CI: 45.2-57.7; 95%PI: 0.25-0.77), weakness/malaise (46.7%; 95%CI: 35.4-58.4; 95%PI: 0.09-0.88), and scalp tenderness (39.1; 95%CI: 35.3-43.1; 95%PI: 0.22-0.59). Positive TAB results were present in 73.8% of patients (95%CI: 68.1-78.8%; 95%PI: 0.35-0.94). The presence of headache (LogOR = -1.11; 95%CI: -1.92 to -0.29) or PMR (-0.71; 95%CI: -1.09 to -0.32) significantly decreases the chance of receiving positive TAB results.

Since there is a greater likelihood of obtaining negative biopsy results, the TAB may not be required when a patient exhibits a headache along with other clinical symptoms that enable them to be diagnosed with GCA.

We present the case of a 70-year-old man with a history of embolic stroke, atrial fibrillation, deep vein thrombosis, and polymyalgia rheumatica who presented as a stroke code with transient right-sided focal neurologic deficits (motor and sensory), mild alteration in consciousness, and mild aphasia. His cerebrovascular imaging revealed new multifocal intracranial stenoses. Despite best medical management, this patient continued to have recurrent symptomatic cerebrovascular events. This case illustrates the evaluation approach and key differential diagnoses to consider in patients with stroke-like symptoms that fail to respond to best stroke medical management. Readers will be taught the importance of considering unusual stroke mechanisms in their initial evaluation of stroke codes and the intricate subtleties in medical and interventional management decisions.

To investigate differences in arterial involvement patterns on 18F-FDG PET-CT between predominant cranial and isolated extracranial phenotypes of giant cell arteritis (GCA).

A retrospective review of 18F-FDG PET-CT findings was conducted on 140 patients with confirmed GCA. The patients were divided into two groups: the cranial group, which presented craniofacial ischemic symptoms either at diagnosis or during follow-up, and the isolated extracranial group which never exhibited such manifestations.

Of the 140 patients (90 women), 99 (71%) were considered to have a predominantly cranial phenotype, while 41 (29%) had isolated extracranial GCA. Patients with the extracranial phenotype were younger (p = 0.001), had lower TAB positivity (25%), and experienced longer diagnostic delays (p = 0.004). Polymyalgia rheumatica was more common in the extracranial group (p = 0.029), which also showed fewer constitutional symptoms, milder increases in acute phase reactants, and more frequent limb claudication and aortic complications, although these differences were not statistically significant. When comparing arterial involvement on 18F-FDG PET-CT, we observed statistically significant differences. The extracranial phenotype showed greater involvement across all segments of the thoracic aorta (p = 0.001), as well as in the abdominal aorta (p = 0.005), subclavian (p = 0.021), iliac (p = 0.004), and femoral arteries (p = 0.025). In contrast, the cranial phenotype exhibited a higher frequency of vertebral artery involvement (p < 0.001).

Significant differences in arterial involvement patterns on 18F-FDG PET-CT were observed between phenotypes. These findings may explain atypical symptoms such as inflammatory lower back pain or limb claudication and the increased risk of aortic complications in extracranial GCA.

Giant cell arteritis (GCA) may present as proximal aortic pathology requiring surgical intervention. We present our experience with surgical management of GCA in patients presenting with proximal aortic disease.

From January 1993 to May 2020, 184 adult patients were diagnosed with GCA on histopathology after undergoing cardiac surgery. Survival was estimated with Kaplan-Meier method. Reoperation rates were estimated with cumulative incidence accounting for competing risks of death.

The most common indication for surgery was ascending aortic aneurysm (n = 179, 97.3%). Stroke occurred in 6 (3.3%), pneumonia in 8 (4.4%), and dialysis in 3 (1.6%) patients. Multivariable analysis found advanced age (hazard ratio [HR], 1.054; 95% confidence interval [CI], 1.026-1.082, P < .001), recent heart failure (HR, 1.890; 95% CI, 1.016-3.516, P = .04), peripheral vascular disease (HR, 2.229; 95% CI, 1.458-3.624, P < .001), and cerebrovascular disease (HR, 1.762; 95% CI, 1.035-3.000, P = .03) as predictors of late mortality. Median follow-up was 13.7 years, and 30-day mortality was 1.5%. Nineteen patients underwent 24 aortic reinterventions including aortic arch reconstruction (n = 4), descending thoracic aorta aneurysm repair (n = 8), thoracoabdominal aortic aneurysm repair (n = 11), and pseudoaneurysm repair (n = 1). Rate of reintervention on the aorta was 3.9% (95% CI, 1.9%-8.1%), 7.1% (95% CI, 4.1%-12.3%), 12.8% (95% CI, 8.3%-19.6%), and 12.8% (95% CI, 8.3%-19.6%) at 1, 5, 10, and 15 years, respectively.

Surgery in patients with GCA can be performed with acceptable early and late outcomes. Advancing age, heart failure, peripheral vascular disease, and cerebrovascular disease are risk factors for worse survival. Postoperative surveillance is important as need for aortic reintervention is not uncommon.

Aortitis in Giant Cell Arteritis (GCA-aortitis) is a frequent complication that may lead to aneurysms. Tocilizumab (TCZ) was approved in GCA, but the efficacy in GCA-aortitis and aneurysms has not been analyzed to date. Our aim was to assess the effectiveness and safety of TCZ in a wide series of GCA-aortitis and aneurysms.

Multicentre observational study with GCA-aortitis treated with TCZ. GCA was diagnosed by: a) ACR criteria, b) temporal artery biopsy, and/or c) imaging techniques. Aortitis was diagnosed mainly by PET/CT. Main outcomes were EULAR and imaging remission. Others were clinical remission, analytical normalization, corticosteroid-sparing effect, and the prevention and improvement of aneurysms.

196 patients with GCA-aortitis treated with TCZ. After 6 months, 72.2% reached EULAR remission but only 12% an imaging remission; increasing up-to 81.4% and 31.8%, respectively, at 24 months. A rapid clinical remission, ESR and CRP normalization was observed in 47.4%, 84.3% and 55.6%, at 1 month, increasing to 89.6%, 85.3% and 80.3% at 24 months, respectively. Aneurysms were present in 10 (5%) patients. Five of them required early surgery, while 3 others enlarged. No patient on TCZ therapy developed aneurysms during follow-up.

In patients with GCA-aortitis treated with TCZ, a rapid and maintained clinical and analytical improvement was observed. However, there was an uncoupling between clinical and EULAR remission with imaging remission.

To identify factors associated with the progression of giant cell arteritis (GCA)-related or associated aortic dilations.

In this retrospective study, 47 GCA patients with aortic dilation were longitudinally analyzed. Each patient underwent ≥2 imaging scans of the aorta during the follow-up. Three progression statuses of aortic dilations were distinguished: fast-progressive (FP) defined by a progression of the aortic diameter ≥5 mm/year or ≥1 cm/2 years, slow progressive (SP) by a progression of the aortic diameter >1 mm during the follow-up, and not progressive (NP) when aortic diameter remained stable.

Among the 47 patients with aortic dilation, the thoracic section was involved in 87 % of patients. Within a total follow-up of 89 [6-272] months, we identified 13 (28 %) patients with FP dilations, and 16 (34 %) and 18 (38 %) patients with SP and NP dilations, respectively. No differences regarding baseline characteristics, cardiovascular risk factors or treatments were observed among the 3 groups. However, FP patients more frequently showed atheromatous disease (p = 0.04), with a more frequent use of statins (p = 0.04) and antiplatelet agents (p = 0.02). Among the 27 (57 %) patients with aortitis, aortic dilation developed on an inflammatory segment in 23 (85 %). Among the FP patients who underwent aortic surgery with available histology (n = 3), all presented active vasculitis.

This study suggests that aortic inflammation, as well as atheromatous disease, might participate in the fast progression of aortic dilation in GCA.

Different types of vasculitis can be distinguished according to the blood vessel's size that is preferentially affected: large-vessel, medium-vessel, and small-vessel vasculitides. Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are the main forms of large-vessel vasculitis, and may lead to lumen narrowing. Clinical manifestations of arterial narrowing on the short- and long term include vision loss, stroke, limb ischemia, and heart failure. Imaging tools are well established diagnostic tests for large-vessel vasculitis and may aid therapy monitoring in selected cases while providing important information regarding the occurrence of vascular damage, tissue and organ complications. This review aims to provide the current status of multimodality imaging for the diagnosis and identification of vascular complications in the field of large vessel vasculitis.

Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects elderly individuals. However, data regarding Korean patients with GCA are scarce owing to its extremely low prevalence in East Asia. This study aimed to investigate the clinical characteristics of Korean patients with GCA and their outcomes, focusing on relapse.

The medical records of 27 patients with GCA treated at three tertiary hospitals between 2007 and 2022 were retrospectively reviewed.

Seventeen (63.0%) patients were females, and the median age at diagnosis was 75 years. Large vessel involvement (LVI) was detected in 12 (44.4%) patients, and polymyalgia rheumatica (PMR) was present in 14 (51.9%) patients. Twelve (44.4%) patients had fever at onset. The presence of LVI or concurrent PMR at diagnosis was associated with a longer time to normalization of the C-reactive protein level (p=0.039) or erythrocyte sedimentation rate (p=0.034). During follow-up (median 33.8 months), four (14.8%) patients experienced relapse. Kaplan-Meier analyses showed that relapse was associated with visual loss (p=0.008) and the absence of fever (p=0.004) at onset, but not with LVI or concurrent PMR.

Concurrent PMR and LVI were observed in approximately half of Korean patients with GCA, and the elapsed time to normalization of inflammatory markers in these patients was longer. The relapse rate in Korean GCA is lower than that in Western countries, and afebrile patients or patients with vision loss at onset have a higher risk of relapse, suggesting that physicians should carefully monitor patients with these characteristics.

Giant cell arteritis (GCA) is characterized by inflammation of large and medium vessels. First-line therapy for the treatment of GCA are glucocorticoids, which are effective while potential adverse effects should be considered, especially during long-term use. The aim was to investigate the incidence of glucocorticoids' adverse effects and potential predictors for them.

138 GCA patients were retrospectively evaluated for newly developed glucocorticoid adverse effects in 2020. Potential predictors, defined as initial glucocorticoid pulse therapy, relapse of GCA and concomitant polymyalgia rheumatica as well as parameters of inflammation and endothelial dysfunction, including pulse-wave velocity and intima-media-thickness, were measured in 2012.

Potential new glucocorticoid adverse effects per patient was 1 (25th-75th 0-3) of which chronic kidney disease progression (29%), bone fractures (23.2%), cataracts (18.1%), dementia, and arterial hypertension (each at 12.3%) were most commonly recorded. Significant associations were found between occurrence of any relapse and new diabetes mellitus and between initial glucocorticoid pulse therapy and new dementia (all with p < 0.05). In multivariate regression analysis, any relapse was a predictor for developing diabetes mellitus (OR 9.23 [95% CI 1.33-64.05], p = 0.025). However, no correlations were observed between endothelial dysfunction or inflammatory parameters and development of new glucocorticoid adverse effects.

GCA relapses may be associated for development of diabetes mellitus potentially by increasing glucocorticoid doses. Parameters of inflammation and endothelial dysfunction are not suited predictors for glucocorticoid adverse effects.

To investigate the hypothesis that a history of PMR is associated with a more severe and damaging disease course in newly diagnosed GCA patients.

This was a retrospective analysis of GCA patients diagnosed between December 2006 and May 2021. We compared vascular ultrasound findings (presence of vasculitis and vascular stenosis) in GCA patients with and without prior PMR.

Forty-nine of 311 GCA patients (15.8%) had prior PMR in a median of 30.6 (IQR 7.1-67.3) months before GCA diagnosis. Patients with prior PMR more often had large vessel vasculitis (LVV) (51.0% vs 25.0%, P < 0.001) and stenosis within the vasculitic segments (18.4% vs 3.1%, P < 0.001) on ultrasound. In multivariable analysis, prior PMR remained significantly associated with LVV (odds ratio 7.65, 95% CI: 2.72, 23.97, P < 0.001). Polymyalgic symptoms at GCA diagnosis in the patients without prior PMR were not associated with a higher prevalence of LVV (P = 0.156).

Patients with a diagnosis of PMR before GCA diagnosis had two times more often large vessel involvement and significant more vasculitic stenoses on ultrasound examination than patients without prior PMR. Pre-existing PMR is an independent risk factor for more extensive and advanced ultrasound findings at GCA diagnosis. The contribution of subclinical vasculitis to disease associated damage should be further studied.

Relapses and late complications remain a concern in giant cell arteritis (GCA). Monitoring strategies are required to effectively tailor treatment and improve patients' outcomes. Current monitoring of GCA is based on clinical assessment and evaluation of traditional inflammatory markers such as C reactive protein and erythrocyte sedimentation rate; however, this approach has limited value in patients receiving interleukin (IL)-6 blocking agents. New blood biomarkers that are less dependent on the IL-6 axis such as IL-23, B cell activating factor, osteopontin and calprotectin have been explored, but none of them has yet accumulated sufficient evidence to qualify as a routine follow-up parameter. Imaging techniques, including ultrasound and 18F-fluorodeoxyglucose positron emission tomography/computed tomography, potentially offer additional insights; however, the choice of the imaging method as well as its interpretation must be investigated further. Future studies are required to investigate the outcome of patients with GCA whose treatment decisions are based on traditional plus novel (laboratory and imaging) biomarkers as compared with those undergoing conventional monitoring strategies.

To evaluate the incidence and outcomes of large artery (LA) involvement among patients with giant cell arteritis (GCA) and to compare LA involvement to non-GCA patients.

The study included Olmsted County, Minnesota, USA residents with incident GCA between 1950 and 2016 with follow-up through 31 December 2020, death or migration. A population-based age-matched/sex-matched comparator cohort without GCA was assembled. LA involvement included aortic aneurysm, dissection, stenosis in the aorta or its main branches diagnosed within 1 year prior to GCA or anytime afterwards. Cumulative incidence of LA involvement was estimated; Cox models were used.

The GCA cohort included 289 patients (77% females, 81% temporal artery biopsy positive), 106 with LA involvement.Reported cumulative incidences of LA involvement in GCA at 15 years were 14.8%, 30.2% and 49.2% for 1950-1974, 1975-1999 and 2000-2016, respectively (HR 3.48, 95% CI 1.67 to 7.27 for 2000-2016 vs 1950-1974).GCA patients had higher risk for LA involvement compared with non-GCA (HR 3.22, 95% CI 1.83 to 5.68 adjusted for age, sex, comorbidities). Thoracic aortic aneurysms were increased in GCA versus non GCA (HR 13.46, 95% CI 1.78 to 101.98) but not abdominal (HR 1.08, 95% CI 0.33 to 3.55).All-cause mortality in GCA patients improved over time (HR 0.62, 95% CI 0.41 to 0.93 in 2000-2016 vs 1950-1974) but remained significantly elevated in those with LA involvement (HR 1.89, 95% CI 1.39 to 2.56).

LA involvement in GCA has increased over time. Patients with GCA have higher incidences of LA involvement compared with non-GCA including thoracic but not abdominal aneurysms. Mortality is increased in patients with GCA and LA involvement highlighting the need for continued surveillance.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.

Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

We aimed to identify associations between patterns of large-vessel lesions of large-vessel giant cell arteritis (LV-GCA) and treatment outcomes.

We extracted data on 68 newly diagnosed patients with LV-GCA from a retrospective, multi-centric, nationwide registry of GCA patients treated with glucocorticoids between 2007 and 2014. Patients with aortic lesions were identified based on the findings from contrast-enhanced computed tomography, magnetic resonance imaging, or positron emission tomography-computed tomography (Group 2, n = 49). Patients without aortic lesions were subdivided into LV-GCA with or without subclavian lesions defined as Group 1 (n = 9) or Group 3 (n = 10), respectively. The primary outcome evaluation was failure to achieve clinical remission by Week 24 and/or relapse within 104 weeks.

The mean age and proportion of patients with cranial lesions and polymyalgia rheumatica in Group 2 were numerically lower than in the other two groups. Large-vessel lesions in Group 3 included carotid, pulmonary, renal, hepatic, or mesenteric lesions. The cumulative rate of poor treatment outcomes >2 years was 11.1%, 55.3%, and 88.0% in Groups 1, 2, and 3, respectively (by Kaplan-Meier analysis). The mean time to poor outcome was significantly different between the groups.

Classification by subclavian and aortic lesions may be useful to determine treatment strategy.

To investigate the clinicopathologic features of patients with giant cell arteritis (GCA) who had thoracic aorta aneurysm or dissection surgery.

Patients who had thoracic aorta surgery between January 1, 2000, and December 31, 2021, at the Mayo Clinic, Rochester, Minnesota, were identified with current procedural terminology (CPT) codes. The identified patients were screened for a prior diagnosis of GCA with diagnostic codes and electronic text search. The available medical records of all the patients of interest were manually reviewed. Thoracic aorta tissues obtained during surgery were re-evaluated in detail by pathologists. The clinicopathologic features of these patients were analyzed. Overall observed survival was compared with lifetable rates from the United States population.

Of the 4621 patients with a CPT code for thoracic aorta surgery, 49 had a previous diagnosis of GCA. Histopathologic evaluation of the aortic tissue revealed active aortitis in most patients with GCA (40/49, 82%) after a median (IQR) of 6.0 (2.6-10.3) years from GCA diagnosis. All patients were considered in clinical remission at the time of aortic surgery. The overall mortality compared to age and sex-matched general population was significantly increased with a standardized mortality ratio of 1.55 (95% CI, 1.05-2.19).

Histopathologic evaluation of the thoracic aorta obtained during surgery revealed active aortitis in most patients with GCA despite being considered in clinical remission several years after GCA diagnosis. Chronic, smoldering aortic inflammation likely contributes to the development of aortic aneurysm and dissection in GCA.

Systemic vasculitides encompass a cluster of autoimmune diseases that affect blood vessels, and are characterized by immune-mediated injury to either small- or large-sized blood vessels. Individuals afflicted with systemic vasculitides experience notable morbidity and mortality attributable to cardiovascular manifestations. Noteworthy among these are ischemic heart disease, venous thromboembolism, aortic involvement, valvular irregularities, myocarditis, and pericarditis. This narrative review investigated and evaluated the prevalent cardiovascular disturbances commonly associated with different types of vasculitides. This review also discusses the mechanisms that underlie these manifestations. It also provides a thorough explanation of the many diagnostic techniques essential for detecting the disease at its occult stage. It is essential for healthcare professionals to have knowledge of the cardiovascular complications caused by vasculitides, as this enables them to promptly recognize these symptoms and employ suitable diagnostic techniques early on. By doing so, timely detection can be ensured, which will subsequently aid in initiating appropriate treatment strategies that are vital for decreasing morbidity and mortality in patients with systemic vasculitides.

Giant Cell Arteritis (GCA) is well known to be a critical ischaemic disease that requires immediate medical recognition to initiate treatment and where one in five people still suffer visual loss. The immunopathophysiology has continued to be characterised, and the influencing of ageing in the development of GCA is beginning to be understood. Recent national and international guidelines have supported the directed use of cranial ultrasound to reduce diagnostic delay and improve clinical outcomes. Immediate high dose glucocorticoids remain the standard emergency treatment for GCA, with a number of targeted agents that have been shown in clinical trials to have superior clinical efficacy and steroid sparing effects. The aim of this review was to present the latest advances in GCA that have the potential to influence routine clinical practice.

Giant Cell Arteritis (GCA) is a heterogenous systemic granulomatous vasculitis involving the aorta and any of its major tributaries. Despite increased awareness of large vessel (LV) involvement, studies reporting incidence, clinical characteristics and complications of large-vessel GCA (LV-GCA) show conflicting results due to inconsistent disease definitions, differences in study methodologies and the broad spectrum of clinical presentations. The aim of this systematic literature review was to better define LV-GCA based on the available literature and identify distinguishing characteristics that may differentiate LV-GCA patients from those with limited cranial disease.

Published studies indexed in MEDLINE and EMBASE were searched from database inception to 7th May 2021. Studies were included if they presented cohort or cross-sectional data on a minimum of 25 patients with LV-GCA. Control groups were included if data was available on patients with limited cranial GCA (C-GCA). Data was quantitatively synthesised with application of a random effects meta-regression model, using Stata.

The search yielded 3488 studies, of which 46 were included. Diagnostic criteria for LV-GCA differed between papers, but was typically dependent on imaging or histopathology. Patients with LV-GCA were generally younger at diagnosis compared to C-GCA patients (mean age difference -4.53 years), had longer delay to diagnosis (mean difference 3.03 months) and lower rates of positive temporal artery biopsy (OR: 0.52 [95% CI: 0.3, 0.91]). Fewer LV-GCA patients presented with cranial manifestations and only 53% met the 1990 ACR Classification Criteria for GCA. Vasculitis was detected most commonly in the thoracic aorta, followed by the subclavian, brachiocephalic trunk and axillary arteries. The mean cumulative prednisolone dose at 12-months was 6056.5mg for LV-GCA patients, relapse rates were similar between LV- and C-GCA patients, and 12% of deaths in LV-GCA patients could be directly attributed to an LV complication.

Patients with LV-GCA have distinct disease features when compared to C-GCA, and this has implications on diagnosis, treatment strategies and surveillance of long-term sequalae.

Research into giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) has become more important in the last few decades. Physicians are facing several challenges in managing the diagnosis, treatment, and relapses of GCA and PMR patients. The search for biomarkers could provide elements to guide a physician's decision. In this review, we aim to summarize the scientific publications about biomarkers in GCA and PMR in the past decade. The first point raised by this review is the number of clinical situations in which biomarkers could be useful: differential diagnosis of either GCA or PMR, diagnosis of underlying vasculitis in PMR, prediction of relapse or complications, disease activity monitoring, choice, and modification of treatments. The second point raised by this review is the large number of biomarkers studied, from common markers like C-reactive protein, erythrocyte sedimentation rate, or elements of blood count to inflammatory cytokines, growth factors, or immune cell subpopulations. Finally, this review underlines the heterogeneity between the studies and proposes points to consider in studies evaluating biomarkers in general and particularly in the case of GCA and PMR.

To compare limited with a more extended ultrasound examination (anteromedial ultrasound, A2-ultrasound) to detect large vessel (LV) involvement in patients with newly diagnosed GCA.

Patients with new-onset GCA were included at the time of diagnosis. All patients were examined using limited ultrasound (ultrasound of the axillary artery as visualized in the axilla) and an extended A2-ultrasound method (which also includes the carotid, vertebral, subclavian and proximal axillary arteries), in addition to temporal artery ultrasound.

One hundred and thirty-three patients were included in the study. All patients fulfilled the criteria according to a proposed extension of the 1990 ACR classification criteria for GCA and had a positive ultrasound examination at diagnosis. Ninety-three of the 133 GCA patients (69.9%) had LV involvement when examined by extended A2-ultrasound, compared with only 56 patients (42.1%) by limited ultrasound (P < 0.001). Twelve patients (9.0%) had vasculitis of the vertebral arteries as the only LVs involved. Five patients (3.8%) would have been missed as having GCA if only limited ultrasound was performed. Forty patients (30.0%) had isolated cranial GCA, 21 patients (15.8%) had isolated large vessel GCA and 72 patients (54.1%) had mixed-GCA.

Extended A2-ultrasound examination identified more patients with LV involvement than the limited ultrasound method. However, extended A2-ultrasound requires high expertise and high-end equipment and should be performed by ultrasonographers with adequate training.

Giant cell arteritis (GCA) is a medical emergency, which can lead to irreversible blindness and other ischaemic vascular events if left untreated. Prompt access to specialist assessment, diagnostics in the form of a fast-track pathway (FTP) and access to appropriate treatment are key factors in preventing morbidity associated with this disease. Recent developments in vascular imaging prompted review of our management of GCA patients. Here, we present the newly implemented FTP in GCA at the University College London Hospital, with added vascular imaging in the form of temporal artery ultrasound (TAUS) and [18F]-fluorodeoxyglucose PET-computed tomography ( 18 F-FDG PET-CT) with temporal artery biopsy. The initial pilot data on the FTP showed a significant negative predictive value of the combined TAUS and 18 F-FDG PET-CT, and the vast majority of cases positive on imaging were confirmed by biopsy. Through the new FTP in GCA, the diagnosis was completed within 48-72 h, compared with the conventional pathway time of up to 2-3 weeks awaiting biopsy results. Prompt and accurate diagnosis of GCA enables commencement of corticosteroid (prednisolone) treatment in the appropriate patient population while avoiding unnecessary steroid exposure and toxicity in GCA-negative patients.

To evaluate the feasibility of tocilizumab tapering and withdrawal in patients with giant cell arteritis (GCA).

GCA patients eligible for tocilizumab were prospectively enrolled. Tocilizumab was administered weekly for the first 12 months, every-other-week for an additional 12 months, then discontinued. Relapses on tocilizumab were managed with temporary increases in systemic glucocorticoids or addition of methotrexate. Primary outcome was relapse-free survival at month 6 after tocilizumab suspension. Relapse-free survival on tocilizumab, imaging response, and adverse events were evaluated.

23 GCA patients were enrolled. Reasons for tocilizumab start were relapse (n = 14), persistence of activity (n = 5), or steroid-related adverse events (n = 4). At tocilizumab start, two patients were on methotrexate, which was maintained. Fourteen patients had extracranial vascular involvement on 18FDG-PET/CT. During the first 12 months, four patients (17%) had clinical relapse. At every-other-week tocilizumab start, all patients were in clinical remission, two patients had active vasculitis on 18FDG-PET/CT; two patients were on steroid therapy, and four patients were taking methotrexate. Two patients (9%) relapsed while on every-other-week tocilizumab. At tocilizumab suspension, no patient was on steroid therapy and no patient had signs of active vasculitis on 18FDG-PET/CT. In the 6 months after tocilizumab discontinuation, six patients (26%) relapsed. No new or unexpected safety findings were identified.

Tocilizumab tapered over a two-year period was effective to induce and maintain remission in GCA. Relapses on tocilizumab were minor and responded to incremental changes in therapy. A significant proportion of patients relapsed in the 6 months after therapy suspension.

Interleukin-6 (IL-6) is a proinflammatory cytokine that plays a key role in the pathogenesis and physiology of inflammatory and autoimmune diseases, such as coronary heart disease, cancer, Alzheimer's disease, asthma, rheumatoid arthritis, and most recently COVID-19. IL-6 and its signaling pathway are promising targets in the treatment of inflammatory and autoimmune diseases. Although, anti-IL-6 monoclonal antibodies are currently being used in clinics, huge unmet medical needs remain because of the high cost, administration-related toxicity, lack of opportunity for oral dosing, and potential immunogenicity of monoclonal antibody therapy. Furthermore, nonresponse or loss of response to monoclonal antibody therapy has been reported, which increases the importance of optimizing drug therapy with small molecule drugs. This work aims to provide a perspective for the discovery of novel small molecule IL-6 inhibitors by the analysis of the structure-activity relationships and computational studies for protein-protein inhibitors targeting the IL-6/IL-6 receptor/gp130 complex.

Takayasu's arteritis (TAK) and giant cell arteritis (GCA) are the 2 most common primary large vessel vasculitides (LVV). They share common vascular targets, clinical presentations, and histopathology, but target a strikingly different patient demographic. While GCA predominantly affects elderly people of northern European ancestry, TAK preferentially targets young women of Asian heritage. Cardiovascular diseases (CVD), including ischemic heart disease, cerebrovascular disease, aortic disease, and thromboses, are significantly increased in LVV. In this review, we will compare and contrast the issue of CVD in patients with TAK and GCA, with respect to prevalence, risk factors, and mechanisms of events to gain an understanding of the relative contributions of active vasculitis, vascular damage, and accelerated atherosclerosis. Controversies and possible mitigation strategies will be discussed.

Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging. Early recognition of LVV and treatment adaptation is essential because vascular complications (aneurysm, dilatations, ischemic complications) or treatment related side effects can occur frequently in these patients. 18-fluorodeoxyglucose positron emission tomography/computed tomography (2-[18F]FDG-PET/CT) is increasingly used to diagnose, follow, and evaluate treatment response in LVV. In this review, we aimed to summarize the current evidence on the value of 2-[18F]FDG-PET/CT for diagnosis, follow, and treatment monitoring in LVV.

Two male patients aged above 70 years were investigated for chronic non-specific symptoms and evidence of significant systemic inflammation, but without classic 'cranial symptoms' of giant cell arteritis (GCA). Each patient had multiple non-diagnostic investigations, but finally extensive large-vessel vasculitis was revealed by whole body positron emission tomography/CT imaging. Both cases were confirmed to have GCA on temporal artery biopsy and responded well to initial high-dose prednisolone therapy. The patients successfully completed 12 months of steroid-sparing therapy with tocilizumab and achieved remission of their condition.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are two common systemic inflammatory conditions with a combined lifetime risk of approximately 3.5% in women and 1.5% in men. They are intimately associated with the aging process, virtually never occurring prior to 50 years of age and becoming more common over time. The reasons for this are unclear, but likely relate in part to factors related to aging of the immune system. The treatment of both GCA and PMR is traditionally based on glucocorticoids, frequently requiring a prolonged treatment course over long periods of time. Other medications are belatedly entering our treatment armamentarium, but their exact place in treatment algorithms remains to be fully defined and it is likely glucocorticoids will remain a cornerstone of our treatment in GCA and PMR for the foreseeable future. As a result, people with GCA and PMR will continue to be exposed to a significant cumulative glucocorticoid burden with all of the attendant potential adverse events, including osteoporosis. The predominantly post-menopausal female population that most commonly develops PMR and GCA is also the population that is most affected by osteoporosis. Given the risk of glucocorticoid-induced osteoporosis and subsequent fragility fractures, a planned treatment approach from glucocorticoid initiation is needed in these conditions. For the majority of patients, this will entail ensuring sufficiency of calcium and vitamin D as well as antiresorptive treatments. In this article, we discuss considerations around optimisation of metabolic bone health in GCA and PMR.

Recent advances in imaging revealed that giant cell arteritis (GCA) is frequently associated with large vessel involvement (LVI), but they may also contribute to earlier diagnosis and treatment of LV-GCA. We aimed to compare the clinical characteristics of GCA with or without LVI and evaluate its association with clinical outcomes.

We retrospectively reviewed the medical records of 36 patients with GCA in Kyoto University Hospital.

Eighteen patients each were assigned to the LVI(+) and LVI(-) groups. Five-year survival rates in the LVI(+) group were better than in the LVI(-) group (p = .034), while five-year relapse-free survival rates were similar between the groups (p = .75). The LVI(+) group required lower doses of glucocorticoid at month 6 (p = .036). Disease activity evaluated with the Birmingham Vasculitis Activity Score at disease onset was higher in the LVI(-) group (p = .014), and the Vasculitis Damage Index score examined at the last visit was higher in the LVI(-) group (p = .011).

GCA without LVI had more active disease, severer vascular damage, and worse survival, possibly because of ophthalmic complications and their greater glucocorticoid requirement. Our results revisit the impact of cranial manifestations on disease severity and morbidity.

Aortic dilatation (AD) occurs in up to 30% of patients with giant cell arteritis (GCA). Reliable biomarkers for AD development, however, are still absent. The aim of this exploratory study was to evaluate whether immunological parameters are associated with the occurrence of AD in GCA. Cross-sectional study on 20 GCA patients with AD, 20 GCA patients without AD, and 20 non-GCA controls without AD measuring leukocytes, neutrophils, lymphocytes, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum amyloid A (SAA), interferon (IFN)-α, IFN-γ, IFN-γ-induced protein 10 (IP-10), interleukin (IL) 5, IL-8, IL-10, IL-17A, IL-18, IL-1 receptor antagonist, tumor necrosis factor (TNF)-α, platelet-derived growth factor (PDGF), L-selectin, P-selectin, and soluble intercellular adhesion molecule 1 (sICAM-1). AD was measured by aortic contrast-enhanced computed tomography and defined by enlargement of the aorta above population-based aortic diameters adjusted by age, gender, and body surface area. No significant differences were observed between GCA patients with AD and GCA patients without AD concerning levels of leukocytes, neutrophils, lymphocytes, CRP, ESR, SAA, IL-8, IL-18, PDGF, IP-10, selectins, and sICAM-1. Values of IFN-α, IFN-γ, IL-5, IL-10, IL-17A, IL-1 receptor antagonist, and TNF-α were all below the detection limits in more than 70% of subjects. Lymphocytes and CRP revealed positive correlations with the diameter of the thoracic descending aorta. Immunological parameters were not useful to conclude on the presence of AD in GCA. Further studies are required to test if CRP and lymphocytes may be useful to predict future development of AD in GCA.

Giant cell arteritis (GCA) is the most common vasculitis affecting people older than 50 years. The last decades have shed new light on the clinical paradigm of this condition, expanding its spectrum beyond cranial vessel inflammation. GCA can be now considered a multifaceted vasculitic syndrome encompassing inflammation of cranial and extra-cranial arteries and girdles, isolated or combined. Such heterogeneity often leads to diagnostic delays and increases the likelihood of acute and chronic GCA-related damage. On the other hand, the approach to suspected GCA patients has been revolutionized by the introduction of vascular ultrasound which allows a rapid, cost-effective, and non-invasive GCA diagnosis. Likewise, the use of tocilizumab is now part of the therapeutic algorithm of GCA and ensures a satisfactory disease control even in steroid-refractory patients. Nonetheless, some aspects of GCA still need to be clarified, including the clinical correlation of different histological patterns, and the prevention of long-term vascular complications. This narrative review depicts the diagnostic and therapeutic aspects of GCA most relevant in clinical practice, with a focus on clinical updates and novelties introduced over the last decade.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate. Structure: Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.

The "2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease" provides recommendations to guide clinicians in the diagnosis, genetic evaluation and family screening, medical therapy, endovascular and surgical treatment, and long-term surveillance of patients with aortic disease across its multiple clinical presentation subsets (ie, asymptomatic, stable symptomatic, and acute aortic syndromes).

A comprehensive literature search was conducted from January 2021 to April 2021, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Library, CINHL Complete, and other selected databases relevant to this guideline. Additional relevant studies, published through June 2022 during the guideline writing process, were also considered by the writing committee, where appropriate.

Recommendations from previously published AHA/ACC guidelines on thoracic aortic disease, peripheral artery disease, and bicuspid aortic valve disease have been updated with new evidence to guide clinicians. In addition, new recommendations addressing comprehensive care for patients with aortic disease have been developed. There is added emphasis on the role of shared decision making, especially in the management of patients with aortic disease both before and during pregnancy. The is also an increased emphasis on the importance of institutional interventional volume and multidisciplinary aortic team expertise in the care of patients with aortic disease.