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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: The aim is to access the real-world clinical management of physicians who treat Takayasu arteritis (TAK) and giant cell arteritis (GCA) after the publication of the Japanese Circulation Society (JCS) 2017 Guidelines for the Management of Vasculitis Syndrome. Methods: This descriptive, cross-sectional study utilized self-administered electronic questionnaires, which were answered in February 2022 by physicians treating TAK or GCA and registered with Macromill Inc. Results: The 329 survey respondents were enrolled. The 2017 JCS Guidelines were the most commonly referenced information source for resolving clinical questions, accessed by 70% of respondents. Ophthalmoscopy was performed in only 50% of patients with TAK and in 70% for GCA. The median percentages of patients who underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography for TAK and GCA patients were 23% and 20% at diagnosis, respectively, and 10% each at follow-up within 12 months. Tocilizumab was the most frequently used medication in combination with glucocorticoids for both TAK and GCA, especially in remission induction therapy for relapsed patients. Conclusions: The majority of physicians treating TAK and GCA referred to the 2017 JCS guidelines. This report clarified the current clinical practice for large vessel vasculitis in Japan, providing information for the next revision of the guidelines. [ABSTRACT FROM AUTHOR]

• In GCA patients with aortic dilation, scarce data exist on the evolution of aortic morphology. • More than 25 % of GCA patients with aortic dilation showed a fast progression of aorta caliber. • More than 90 % of the aortic dilations on the abdominal portion of the aorta progressed. • Among patients with aortitis, aortic dilation developed on an inflammatory segment in 85 % of them. • Available histologies of patients with fast progression and aortic surgery showed active vasculitis. 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. [ABSTRACT FROM AUTHOR]

This study examines the characteristics of patients with giant cell arteritis (GCA), the utilization of imaging in GCA diagnosis, and variations in GCA management among specialties. Subjects were identified from the Dallas VAMC database spanning 2010 to 2021 using ICD-9/10 codes for GCA and polymyalgia rheumatica, and a list of temporal artery biopsies (TAB). Patients lacking sufficient data to meet the ACR 1990 classification criteria for GCA were excluded. Categorical variables were compared using Fisher's exact test. Continuous variables were analyzed with the Kruskal–Wallis test. Among 209 identified patients, 41 were excluded due to insufficient data for ACR classification. The cohort comprised 91.9% males with a median age of 69. Of the remaining 168 patients, 42 received a final diagnosis of GCA, and 15 of these were confirmed with a positive TAB. The most reported initial symptoms were visual disturbances (75.5%) and headaches (67.7%). Ophthalmology was the initial physician for 46% of patients. GCA correlated with co-existing autoimmune diseases, glucocorticoid-sparing treatments, and consultation with a rheumatologist (p < 0.05). There were no significant differences in clinical features or management of the positive and negative TAB GCA groups. GCA presents with heterogeneous symptoms making diagnosis challenging. Scalp tenderness and headaches were significantly higher in GCA patients, but sub-group analysis revealed no significant differences among GCA patients. Vascular assessments and adjunct imaging modalities are underutilized. The establishment of multidisciplinary or fast-track clinics may enhance the optimization of GCA management. Key Points • The most common presenting symptoms were blurry vision/visual loss (75.5%), headache (67.7%), and scalp tenderness (35.9%) in descending order. • In sub-group analysis, no significant differences were found between GCA sub-groups, but when compared to the non-GCA group, were found to have significantly higher rates of headache and scalp tenderness. • Compared to other specialties, rheumatologists were more likely to use advanced imaging, and to prescribe glucocorticoid-sparing treatments. • Systematic and comprehensive assessment and multidisciplinary approach could improve diagnosis and management. [ABSTRACT FROM AUTHOR]

Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65–0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06–1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis. [ABSTRACT FROM AUTHOR]

Summary Large vessel vasculitides (LVV) such as giant cell arteritis, Takayasu arteritis and aortitis/periaortitis are characterised by immune‐mediated inflammation of medium to large arteries. Clinical disease manifestations can be non‐specific and diagnostic imaging plays an important role in the diagnostic pathway. In recent years, FDG PET/CT has proven to be a powerful metabolic tool that can provide a wholed body, non‐invasive assessment of vascular inflammation. This review outlines the clinical features of large vessel vasculitis and the closely related entity of polymyalgia rheumatica, summarises the evidence for FDG PET/CT in the assessment of these conditions, and provides guidance for patient preparation, image acquisition and interpretation. [ABSTRACT FROM AUTHOR]

Background/Objectives: Imaging studies have transformed the diagnosis of large vessel vasculitis (LVV) involvement in giant cell arteritis (GCA). A positron emission tomography/computed tomography (PET/CT) scan with 18-fluorodeoxyglucose (18F-FDG) has emerged as a valuable tool for assessing LVV. We aimed to determine the utility of an 18F-FDG-PET/CT scan in detecting LVV in GCA in the ARTESER registry. Methods: The ARTESER study is a large multicenter, retrospective, longitudinal, and observational study, promoted by the Spanish Society of Rheumatology. It included patients newly diagnosed with GCA across 26 tertiary hospitals from 1 June 2013 to 29 March 2019. Patients with a diagnosis of incidental GCA were included if they fulfilled specific criteria, including the ACR 1990 criteria, positive imaging examinations, or the expert clinical opinion of investigators. Differences between patients with positive and negative 18F-FDG-PET/CT scan results were analyzed using a bivariate model. A regression model assessed associations in patients with a positive scan, and the predictive capacity of the cumulative dose of glucocorticoids (GC) on PET scan outcomes was evaluated using ROC curve analysis. Results: Out of 1675 GCA patients included in the registry, 377 met the inclusion criteria of having an 18F-FDG-PET/CT scan. The majority were diagnosed with a cranial GCA phenotype, and 65% had LVV. The thoracic aorta was the most frequently affected. Cardiovascular disease, diabetes, and older age had a negative association with a positive scan outcome. The OR for having a positive 18F-FDG-PET/CTC scan was lower as the number of days increased. Depending on the cumulative dosage of the GC, the 18F-FDG-PET/CT scan showed an AUC of 0.74, with a Youden index > 60 mg/day. Conclusions: Younger patients showed a higher probability of presenting LVV as detected by the 18F-FDG-PET/CT scan. The timing of the examination and the cumulative dosage of the GC influenced the likelihood of a positive result, with earlier tests being more likely to detect inflammation. [ABSTRACT FROM AUTHOR]

Introduction: Calciphylaxis is a condition that causes vascular calcification and intimal proliferation with thrombotic occlusion of small-to-medium-sized vessels. Case Presentation: We describe a case of a 64-year-old woman who presented with a clinical picture that was suggestive of anterior arteritic ischemic optic neuropathy due to giant cell arteritis (GCA), but was found to have calciphylaxis confirmed by histologic examination. When calciphylaxis affects the blood supply to the eye, commonly it causes sudden vision loss, pallid optic disk edema, and a relative afferent pupillary defect, all of which were observed in our patient. Conclusion: There is no known cure for calciphylaxis, while in GCA, high-dose corticosteroid therapy is essential to prevent involvement of the contralateral eye. As the management approaches for these two diseases are different, it is important to have performed a thorough clinical examination along with detailed histopathological testing to rule out calciphylaxis of the temporal artery in patients suspected to have GCA. [ABSTRACT FROM AUTHOR]

Background: Takayasu arteritis (TAK) and giant cell arteritis (GCA) are two major large vessel vasculitis, with varied epidemiology by geographical location, age, and race. However, the epidemiological data in Chinese population is rarely reported. This study estimated the epidemiology of TAK and GCA in Shanghainese individuals residing in China over a 10‐year period. Methods: TAK data for individuals over 16 years and GCA data for individuals over 50 years were retrieved from 38 comprehensive hospitals in Shanghai, China through the electronic medical record systems between January 1, 2011, and December 31, 2020. A systematic literature review was performed to determine the global distribution of TAK and GCA by searching PubMed, Ovid‐Medline, Excerpta Medica Database (EMBASE), and Web of Science. Results: In 173 identified TAK cases (67% females; mean age, 46 ± 15 years), the period prevalence was 11.72 cases per million, and the mean annual incidence was 1.33 cases per million. The highest prevalence (17.74 cases per million) and incidence (1.71 cases per million) were observed in the 16‐ to 34‐year‐old age group. In 92 identified GCA cases (56% females; age, >50 years), the period prevalence was 2.73 cases per 100 000 persons, and the mean annual incidence was 1.91 cases per 100 000 persons. Meta‐analysis of the incidence study of TAK and GCA showed that the pooled incidence rate of TAK and GCA was 1.29 per million and 15.48 per 100 000 person‐years, respectively. Subgroup analysis showed that the incidence of TAK was significantly higher in Asia than in other regions, while the incidence of GCA was higher in Europe, especially North Europe. Conclusion: The epidemiological patterns of TAK and GCA were comprehensively mapped globally and locally, in Shanghai, China. [ABSTRACT FROM AUTHOR]

Objectives Our aim was to introduce a standardized system for assessing the extent of GCA on MRI, i.e. the Magnetic Resonance Vasculitis Activity Score (MRVAS). To obtain a comprehensive view, we used an extensive MRI protocol including cranial vessels and the aorta with its branches. To test reliability, MRI was assessed by four readers with different levels of experience. Methods A total of 80 patients with suspected GCA underwent MRI of the cranial arteries and the aorta and its branches (20 vessel segments). Every vessel was rated dichotomous [inflamed (coded as 1) or not (coded as 0)], providing a summed score of 0–20. Blinded readers [two experienced radiologists (ExR) and two inexperienced radiologists (InR)] applied the MRVAS on an individual vessel and an overall level (defined as the highest score of any of the individual vessel scores). To determine interrater agreement, Cohen's κ was calculated for pairwise comparison of each reader for individual vessel segments. Intraclass correlation coefficients (ICCs) were used for the MRVAS. Results Concordance rates were excellent for both subcohorts on an individual vessel-based (GCA: ICC 0.95; non-GCA: ICC 0.96) and overall MRVAS level (GCA: ICC 0.96; non-GCA: ICC 1.0). Interrater agreement yielded significant concordance (P  < 0.001) for all pairs (κ range 0.78–0.98). No significant differences between ExRs and InRs were observed (P  = 0.38). Conclusion The proposed MRVAS allows standardized scoring of inflammation in GCA and achieved high agreement rates in a prospective setting. [ABSTRACT FROM AUTHOR]

Objectives To elucidate the association between genetic variants and the risk of GCA via large-scale genome-wide association studies (GWAS). In addition, to assess the causal effect of a specific molecule by employing the obtained GWAS results as genetic epidemiological tools. Methods We applied additional variant quality control to the publicly available GWAS results from the biobanks of the UK (UKBB) and Finland (FinnGen), which comprised 532 cases vs 408 565 controls and 884 cases vs 332 115 controls, respectively. We further meta-analysed these two sets of results. We performed two-sample Mendelian randomization (MR) to test the causal effect of low-density lipoprotein (LDL) cholesterol on the risk of GCA. Results The MHC class II region showed significant associations in UKBB, FinnGen and the meta-analysis. The VLDLR region was associated with GCA risk in the meta-analysis. The T allele of rs7044155 increased the expression of VLDLR, decreased the LDL cholesterol level and decreased the disease risk. The subsequent MR results indicated that a 1 s. d. increase in LDL cholesterol was associated with an increased risk of GCA (odds ratio 1.21, 95% CI 1.01–1.45; P  = 0.04). Conclusions Our study identified associations between GCA risk and the MHC class II and VLDLR regions. Moreover, LDL cholesterol was suggested to have a causal effect on the risk of developing GCA. [ABSTRACT FROM AUTHOR]

Objectives We investigated the potential of serum proteins for distinguishing clinical and molecular subtypes in patients with GCA. Methods Proximity extension assays were used to analyse 1463 proteins in serum samples from patients with new-onset GCA (n  = 16) and patients who have achieved remission (n  = 13). Unsupervised and supervised cluster analyses were performed. Results Unsupervised cluster analysis identified three distinct clusters based on the protein signature. Compared with cluster 2, patients of cluster 1 had fewer PMR symptoms, increased levels of macrophage migration inhibitory factor (MIF) and pronounced NF-κB, STAT5 and IL-1 signalling. The changes in serum proteins upon remission differed between cluster 1 and 2. Patients with cranial GCA were characterized by altered endothelial and Th17 signalling, whereas patients not responding to treatment within the GUSTO-trial showed increased Th1 and diminished B cell signalling. Patients with anterior ischaemic optic neuropathy displayed higher levels of CHI3L1 (YKL40) and MMP12, and reduced levels of TIMP3. Conclusion Protein profiling identified patient clusters in GCA with distinct proteomic features and therefore likely different pathophysiology. These unique proteomic footprints might lead to more targeted treatments in future. [ABSTRACT FROM AUTHOR]

Introduction/Objectives: Optic nerve sheath (ONS) enhancement using magnetic resonance imaging of the orbits was observed in patients with giant cell arteritis (GCA). We previously showed that ONS diameter (ONSD) by bedside ultrasound is increased in patient with active GCA. This study aims to assess whether ONSD decreases with clinical remission in patients with GCA. Methods: A prospective cohort study was conducted from June 2022 to January 2023. Patients who had an optic nerve ultrasound at GCA diagnosis as part of a previous crosssectional study were eligible. Optic nerve ultrasound was performed by the same investigator at diagnosis and month 3. ONSD (includes the optic nerve and its sheath) and optic nerve diameter (OND) were measured. Descriptive statistics for baseline characteristics and paired sample t-test were performed to assess the mean difference in OND and ONSD between diagnosis and month 3. Results: Nine patients with GCA were included. The median age at disease onset was 79 years (interquartile range (IQR) of 79–82 years), and 7 patients were males. All patients were in clinical remission at month 3 on prednisone (median dose of 15 mg/day, IQR of 10–25 mg). The mean ONSD was lower at month 3 (3.76 mm) compared to baseline (5.98 mm), with a paired mean difference of 2.22 mm (95% CI 1.41–3.03 mm, p < 0.001). As anticipated, OND measurements did not vary between diagnosis and month 3. Conclusion: ONSD on ultrasound improves after 3 months of therapy in patients with GCA. A longer prospective study is required to determine if ONSD is useful to assess disease activity in GCA. Key Points • ONS ultrasound can identify patients with active GCA. • The ONSD on ultrasound is dynamic and improved after 3 months of GCA therapy. • ONS ultrasound may be useful to monitor disease activity in GCA. [ABSTRACT FROM AUTHOR]

La pseudopolyarthrite rhizomélique, qui se manifeste essentiellement par des douleurs inflammatoires des ceintures après 50 ans, peut être associée ou non à une artérite à cellules géantes crânienne ou extracrânienne qui survient sur le même terrain. Les deux pathologies touchent les deux sexes et toutes les ethnies mais sont plus fréquentes chez les femmes caucasiennes. Leur coût dépend plus des effets secondaires des corticoïdes que du coût d'achat des traitements. Ce coût est plus élevé en cas d'artérite à cellules géantes associée, surtout du fait du bilan initial plus important (exploration vasculaire) et des complications (car la corticothérapie est plus forte). Si le coût des corticoïdes est très faible, l'ajout d'un biologique prescrit pour corticodépendance l'augmente considérablement sans qu'il soit facile d'en mesurer le bénéfice eu égard au caractère très décalé dans le temps des complications de la corticothérapie. Polymyalgia rheumatica is mainly manifested by inflammatory pains of the belts after 50 years. It may or may not be associated with giant cell arteritis that may present as a cranial or extracranial arteritic pathology. Both pathologies affect both sexes and all ethnicities but are more common in Caucasian women. The cost of the disease is higher in cases of giant cell arteritis, especially because of the initial larger assessment (vascular exploration) and complications (because corticosteroid therapy is stronger). The cost of drugs is very low unless a biologic is prescribed, which then significantly increases the costs; it is uneasy to measure their benefit given the very staggered nature of the cost of complications of corticosteroid therapy. [ABSTRACT FROM AUTHOR]

Inflammatory causes of stroke are frequent and often pose diagnostic and therapeutic challenges due to the scarcity of randomized trials and the absence of clear guideline recommendations for many scenarios. Following the publication of the recommendations of the European Stroke Organization on primary angiitis of the central nervous system (PACNS) last year, the German Neurological Society (DGN) has issued very clear guidelines this year on the diagnostics and treatment of PACNS and updated the recommendations for systemic vasculitides; however, stroke often occurs not only as a result of primary vascular inflammation but also as a complication of another organ infection. Approximately 5% of all patients with sepsis, ca. 20% of patients with bacterial meningitis and up to 40% of patients with bacterial endocarditis suffer from a stroke as a complication. This article summarizes the key characteristics of these inflammatory causes of stroke and particularly focuses on the current recommendations for diagnostic and therapeutic management. [ABSTRACT FROM AUTHOR]

Purpose: To describe the demographic and clinical characteristics of uveitis in patients with giant cell arteritis (GCA), their treatments, and evolution. Methods: A national retrospective cohort study was performed. The inclusion criteria were as follows: patients with GCA fulfilling the 2022 ACR/EULAR criteria and a diagnostic of uveitis attested by an ophthalmologist. Results: Seven women were included. The median age at diagnosis of uveitis was 71 years (64–84). All uveitis were diagnosed during active GCA (five at initial diagnosis, two at relapse). All uveitis were acute (100%), mostly anterior (86%) and bilateral (71%). Granulomatous features were less common (29%). All uveitis were treated with local and systemic corticosteroids. After a median follow-up of 30 (21–55) months, all patients achieved complete ophthalmic remission, with only one relapse at 2 years. GCA was also in complete remission. Conclusions: Uveitis could reveal GCA, and its presence correlated with disease activity of GCA. The most frequent clinical presentation of uveitis was acute and anterior; using local and systemic corticosteroids, the prognosis was favorable. [ABSTRACT FROM AUTHOR]

Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab. A lady in her 80s with past medical history of well-controlled hypertension, bladder cancer in remission, a twenty-pack year smoking history, cervical and lumbar spine stenosis, and recent immunizations presented with acute onset of right-sided pupil-sparing third nerve palsy. Labs were pertinent for an elevated ESR and CRP. Brain imaging was without acute abnormalities. A temporal artery biopsy established evidence of healed arteritis and a diagnosis of GCA was made. The patient was treated with pulse-dose steroids followed by an oral steroid taper and tocilizumab. At one month follow-up, there was partial resolution in her ophthalmoplegia. We underscore the importance of considering temporal arteritis as a potential cause of third nerve palsy in the elderly before attributing it solely to microvascular ischemia, particularly in patients with constitutional features. Additionally, in our comprehensive literature review, we aim to consolidate the existing data from similar presentations, shedding light on the clinical manifestation and disease trajectory. [ABSTRACT FROM AUTHOR]

Objective: Signal regulatory protein α (SIRPα) is found primarily on myeloid cells, including macrophages and neutrophils; binds to CD47; and regulates phagocytosis, antigen presentation, cellular fusion, cell proliferation, and migration. Therefore, SIRPα may be involved in the pathogenesis of autoimmune diseases, including systemic vasculitis. This study aimed to assess SIRPα expression in tissue samples from patients with vasculitis. Methods: Immunohistochemical staining for SIRPα was performed on temporal artery (TA), kidney, and lung biopsy samples from patients with giant cell arteritis (GCA), patients with microscopic polyangiitis (MPA), patients with granulomatosis with polyangiitis (GPA), and patients without vasculitis. A score of SIRPα+ expression was calculated, derived from the percentages of monocytes, macrophages, and dendritic cells and neutrophils with different staining intensities in affected tissues. Results: A total of 46 samples from patients with different vasculitides (GCA, MPA, and GPA) were included in the study. Tissue samples included TA samples from 15 patients with GCA; kidney samples from 11 and 9 patients with GPA and MPA, respectively; and lung samples from 11 patients with GPA. Most tissue samples from patients with active vasculitis (15 of 15 TA samples, 17 of 20 kidney samples, and 9 of 11 lung samples) showed SIRPα staining. SIRPα staining intensity was less in kidney samples compared to TA and lung samples. Conclusion: This study demonstrates high‐level expression of SIRPα in macrophages and monocytes in affected tissue in systemic vasculitis. These findings provide a foundation for further studies exploring the role of the SIRPα–CD47 pathway in the pathogenesis of systemic vasculitis and the potential for the blockade of SIRPα and/or the depletion of SIRPα+ cells as treatment of systemic vasculitis. [ABSTRACT FROM AUTHOR]

The ultrasonic diagnostic equipment for ophthalmology includes conventional ultrasonography, high-frequency ultrasonography, ultrasound biomicroscopy and Color-Doppler ultrasonography, which is of great significance for the diagnosis and differential diagnosis of various ophthalmic diseases. The progress of ultrasound diagnosis, especially the application of high-frequency ultrasonography and Color-Doppler ultrasonography in ophthalmology has promoted the progress of diagnosis of optic disc edema, cranial hypertension, carotid cavernous fistula, ocular ischemia syndrome and giant cell arteritis and other complex neurological ophthalmic diseases. [ABSTRACT FROM AUTHOR]

This article discusses the case of a 58-year-old man who presented with subacute digital ischemia of all limbs. The patient had a history of Raynaud phenomenon and worked in a cold environment. The article describes the patient's symptoms, physical examination findings, and laboratory results. The most likely diagnosis is determined to be thromboangiitis obliterans (TAO), a nonatherosclerotic and segmental inflammatory disease that affects the small- and medium-sized arteries and veins of the extremities. The article emphasizes the importance of complete abstinence from tobacco products as the cornerstone of therapy for TAO, as smoking cessation is the only reliable way to prevent disease progression and decrease the risk of amputation. Other treatment options include dihydropyridine calcium channel blockers and intravenous alprostadil. Revascularization may be considered if ischemic ulcers or gangrene are present, but it is often not feasible due to the diffuse involvement of distal arteries. [Extracted from the article]