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Orofacial pain is a worldwide pain problem, with many patients unable to find appropriate diagnosis and treatment. Orofacial pain includes pain arising from the odontogenic and nonodontogenic structures in the head and neck region. Dental clinicians need to have a thorough knowledge and skill to diagnose, manage, and treat patients with odontogenic pain or refer patients for treatment of nonodontogenic pain to specialists such as orofacial pain specialists, neurologists, otolaryngologists, and rheumatologists. More often, dental practitioners diagnose patients with a temporomandibular disorder (TMD), and when treatment is ineffective, term it "atypical facial pain." The first requirement for effective treatment is an accurate diagnosis. Dental clinicians must be aware of giant cell arteritis (GCA), a chronic large-vessel vasculitis, primarily affecting adults over the age of 50 years, as it frequently mimics and is misdiagnosed as TMD. GCA is associated with loss of vision, and stroke and can be a life-threatening disorder. Therefore, diagnostic testing for GCA and differential diagnosis should be common knowledge in the armamentarium of all dental clinicians. Historically, temporal artery biopsy was considered the definitive diagnostic test for GCA. Temporal artery ultrasound (TAUSG), a safe and noninvasive imaging modality, has replaced the previous diagnostic gold standard for GCA, the temporal artery biopsy, owing to its enhanced diagnostic capabilities and safety profile. The present case report describes a patient with GCA, and the role TAUSG played in the diagnosis. Case report: A 72-year-old woman presented with left-sided facial pain, jaw claudication, dysesthesia of the tongue, and episodic loss of vision of 2 years' duration. She was diagnosed with and treated for a myriad of dental conditions including endodontia and temporomandibular joint therapy with no benefit. A thorough history and physical examination, combined with serologic analysis, led to the diagnosis of GCA and TAUSG, which confirmed the diagnosis. Conclusion: This report underscores the responsibility of differential diagnosis and early recognition of GCA facilitated by TAUSG in optimizing treatment outcomes as a viable, noninvasive diagnostic tool. [ABSTRACT FROM AUTHOR]

OBJECTIVE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

OBJECTIVES: To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). METHODS: A systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases. Based on evidence and expert opinion, the task force (29 participants from 10 countries consisting of physicians, a healthcare professional and a patient) developed recommendations, with consensus obtained through voting. The final level of agreement was provided anonymously. RESULTS: Five overarching principles and six-specific recommendations were formulated. Management of GCA and PMR should be based on shared decisions between patient and physician recognising the need for urgent treatment of GCA to avoid ischaemic complications, and it should aim at maximising health-related quality of life in both diseases. The treatment targets are achievement and maintenance of remission, as well as prevention of tissue ischaemia and vascular damage. Comorbidities need to be considered when assessing disease activity and selecting treatment. CONCLUSION: These are the first T2T recommendations for GCA and PMR. Treatment targets, as well as strategies to assess, achieve and maintain these targets have been defined. The research agenda highlights the gaps in evidence and the need for future research.

Objectives To examine the clinicopathologic features of patients with polymyalgia rheumatica (PMR) who had thoracic aorta repair surgery. Findings were compared with those of a cohort of patients with giant cell arteritis (GCA) requiring thoracic aorta repair. Methods All patients evaluated at Mayo Clinic in Rochester, MN, with Current Procedural Terminology (CPT) codes for thoracic aorta repair surgery between 2000–2021 were identified. All patients were screened for prior PMR diagnosis. Patients with PMR and no signs of GCA were categorized as clinically isolated PMR. The medical records of all patients were manually reviewed, and pathologists re-examined all the aortic tissues. Results Of the 4621 patients with at least one CPT code for thoracic aorta repair surgery, 43 patients were diagnosed with clinically isolated PMR before the surgery. Detailed histopathological examination of the aortic tissues revealed active inflammation in 30/43 (70%) patients after a median (IQR) of 10.0 (4.7–13.3) years from the PMR diagnosis. When compared with aortic tissue from patients with a prior diagnosis of GCA, the aorta of patients with PMR had more severe inflammation (Grade 3: 15/30 [50%] vs 5/34 [15%], P  = 0.002). Patients with PMR and thoracic aorta repair may experience a 40% increased risk of mortality compared with the general population, but this did not reach statistical significance (standardized mortality ratio: 1.40; 95% CI: 0.91–2.07). Conclusions Some patients with PMR have subclinical aortic inflammation that is detectable many years after initial diagnosis and may contribute to the development of aortic aneurysm. [ABSTRACT FROM AUTHOR]

Objectives The National Health Service in England funds 12 months of weekly s.c. tocilizumab (qwTCZ) for patients with relapsing or refractory GCA. During the coronavirus disease 2019 (COVID-19) pandemic, some patients were allowed longer treatment. We sought to describe what happened to patients after cessation of qwTCZ. Methods Multicentre service evaluation of relapse after stopping qwTCZ for GCA. The log-rank test was used to identify significant differences in time to relapse. Results A total of 336 GCA patients were analysed from 40 centres, treated with qwTCZ for a median [interquartile range (IQR)] of 12 (12–17) months. At time of stopping qwTCZ, median (IQR) prednisolone dose was 2 (0–5) mg/day. By 6, 12 and 24 months after stopping qwTCZ, 21.4%, 35.4% and 48.6%, respectively, had relapsed, requiring an increase in prednisolone dose to a median (IQR) of 20 (10–40) mg/day. 33.6% relapsers had a major relapse as defined by EULAR. Time to relapse was shorter in those that had previously also relapsed during qwTCZ treatment (P  = 0.0017), in those not in remission at qwTCZ cessation (P  = 0.0036) and in those with large vessel involvement on imaging (P  = 0.0296). Age ≥65 years, gender, GCA-related sight loss, qwTCZ treatment duration, TCZ taper, prednisolone dosing and conventional synthetic DMARD use were not associated with time to relapse. Conclusion Up to half our patients with GCA relapsed after stopping qwTCZ, often requiring a substantial increase in prednisolone dose. One-third of relapsers had a major relapse. Extended use of TCZ or repeat treatment for relapse should be considered for these patients. [ABSTRACT FROM AUTHOR]

Objectives GCA is an inflammatory disease following a chronic, relapsing course. The metabolic alterations related to the intense inflammatory process during the active phase and the rapid impact of steroid treatment remain unknown. This study aims to investigate the serum metabolome in active and inactive disease states. Methods A total of 110 serum samples from 50 patients (33 GCA and 17 PMR) at three time points—0 (V1: active disease), 1 and 6 months (V2 and V3: remission)—of treatment with glucocorticoids (GCs) were subjected to NMR-based metabolomic analysis. Multi- and univariate statistical analyses were utilized to unveil metabolome alterations following treatment. Results Distinct metabolic profiles were identified between activity and remission, independent of disease type. N-acetylglycoproteins and cholines of bound phospholipids emerged as predictive markers of disease activity. Altered levels of 4 of the 21 small molecules were also observed, including increased levels of phenylalanine and decreased glutamine, alanine and creatinine in active disease. Metabolic fingerprinting discriminated GCA from PMR in remission. GCA and PMR patients exhibited characteristic lipid alterations as a response and/or adverse effect of GC treatment. Correlation analysis showed that several identified biomarkers were further associated with acute phase reactants, CRP and ESR. Conclusion The NMR profile of serum metabolome could identify and propose sensitive biomarkers of inflammation. Metabolome alterations, following GC treatment, could provide predictors for future steroid-induced side effects. [ABSTRACT FROM AUTHOR]

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

This article discusses a case of Occult Giant Cell Arteritis (GCA), a rare variant of GCA that primarily affects the eyes. The patient, an 82-year-old Scandinavian woman, presented with visual disturbances and was initially diagnosed with non-arteritic anterior ischemic optic neuropathy (NA-AION). However, further evaluation revealed bilateral optic disc swelling and halo-signs in the temporal arteries, confirming the diagnosis of GCA. Treatment with corticosteroids led to stabilization of the patient's vision. The article emphasizes the importance of ultrasound examinations in detecting Occult GCA, especially in asymptomatic patients or those at risk due to age or demographic factors. [Extracted from the article]

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Giant cell arteritis (GCA) is an autoimmune/autoinflammatory disease affecting large vessels in patients over 50 years old. The disease presents as an acute inflammatory response with two phenotypes, cranial GCA and large-vessel vasculitis (LV)-GCA, involving the thoracic aorta and its branches. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET-CT) is among the imaging techniques contributing to diagnosing patients with systemic disease. However, its association with soluble inflammatory markers is still elusive. This proof-of-concept study aims to identify novel soluble serum biomarkers in PET/CT-positive patients with LV-GCA and associate them with active (0 months) and inactive disease (6 months following treatment), in sequential samples. The most-diseased-segment target-to-background ratio (TBRMDS) was calculated for 13 LV-GCA patients, while 14 cranial GCA and 14 Polymyalgia Rheumatica patients with negative initial PET/CT scans served as disease controls. Serum macrophage-related cytokines were evaluated by cytometric bead array (CBA). Finally, previously published NMR/metabolomics data acquired from the same blood sampling were analyzed along with PET/CT findings. TBRMDS was significantly increased in active versus inactive disease (3.32 vs. 2.65, p = 0.006). The analysis identified nine serum metabolites as more sensitive to change from the active to inactive state. Among them, choline levels were exclusively altered in the LV-GCA group but not in the disease controls. Cytokine levels were not associated with PET/CT activity. Combining CRP, ESR, and TBRMDS with choline levels, a composite index was generated to distinguish active and inactive LV-GCA (20.4 vs. 11.62, p = 0.001). These preliminary results could pave the way for more extensive studies integrating serum metabolomic parameters with PET/CT imaging data to extract sensitive composite disease indexes useful for everyday clinical practice. [ABSTRACT FROM AUTHOR]

The editorial in the Journal of Clinical Medicine discusses the evolving field of vasculitis, highlighting unmet clinical needs in diagnosis and treatment. The Special Issue aims to advance knowledge through innovative research, with articles covering diverse topics like biomarkers and disease mimics. The editorial emphasizes the importance of further research in areas like Giant Cell Arteritis, Takayasu Arteritis, Polyarteritis Nodosa, and ANCA-Associated Vasculitis, calling for improved diagnostic tools, validated biomarkers, and tailored treatment approaches. The authors encourage multi-center collaborations and the integration of emerging technologies to address current limitations and enhance future research in vasculitis. [Extracted from the article]

Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems. It presents a diagnostic and therapeutic challenge due to its broad clinical manifestations. Vasculitis is classified based on the size of the affected vessels: small, medium, large, or variable-sized. Large vessel vasculitis (LVV), particularly giant cell arteritis (GCA) and Takayasu arteritis (TAK), has garnered attention due to its significant morbidity and mortality. Both conditions involve immune-mediated inflammation of the vascular wall, despite differing in epidemiology and presentation. Early identification is crucial to prevent complications like organ ischemia and hemorrhage. Diagnostic accuracy can be hampered by false negative results, making comprehensive investigation essential. Vascular imaging, including computed tomography angiography (CTA), ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT), is key in diagnosing vasculitis, revealing vessel wall thickening and other suggestive features. This article reviews typical and atypical CT and CTA findings in LVV, discusses imaging modalities, and highlights their role in therapeutic management and prognosis. It emphasizes the importance of a multidisciplinary approach and the critical role of radiologists in improving patient outcomes in LVV. [ABSTRACT FROM AUTHOR]

Objective: Inflammation markers, e.g. C- reactive protein (CRP) and sedimentation rate, can be normal despite active vasculitis. Von Willebrand factor (vWF) is secreted from endothelial cells in response to vascular damage. Some reports suggest increased vWF levels in vasculitis. This study aimed to evaluate vWF serum concentration in vasculitis patients as a possible biomarker of disease activity and to review the current literature. Method: Adult patients with systemic vasculitis were prospectively enrolled. Disease activity was recorded using the Birmingham Vasculitis Activity Score (BVAS) version 3. Blood group-adjusted vWF antigen serum level was evaluated at diagnosis and, when available, after treatment. Results: Twenty-five vasculitis patients were compared to 15 healthy controls. The mean age of patients was 56 ± 17 years and 56% were women. Forty percent had anti-neutrophil cytoplasmic autoantibody-associated vasculitis, 20% giant cell arteritis, 16% polyarteritis nodosa, 8% Takayasu arteritis, and the rest had other vasculitides. The mean disease duration was 3.4 ± 4.8 years. Mean vWF was higher in patients with active vasculitis than in healthy controls (212 ± 81% vs 106 ± 26%, p < 0.001). vWF levels directly correlated with BVAS. In 13 patients with active vasculitis who reached remission or low disease activity after treatment, vWF level at follow-up decreased significantly. In three out of five patients who were treated with interleukin-6 inhibitors, vWF was elevated despite normal CRP levels, while vasculitis was clinically active. Conclusion: vWF antigen serum level is increased in active vasculitis and could potentially serve as a biomarker for active disease. [ABSTRACT FROM AUTHOR]