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Abstract Background This report presents the management of patient with extracranial internal carotid artery pseudoaneurysm due to giant cell arteritis. Case presentation Left internal carotid artery pseudoaneurysm was diagnosed in a 57-year-old Ukrainian woman, which became a direct indication for surgical treatment involving aneurysm resection and internal carotid artery reimplantation. The used reconstruction technique with oblique cutting of internal carotid artery, aneurysm resection, ellipse-form anastomosis formation, and distal intima fixation prevents the dissection, restenosis, and aneurysm of anastomosis in the long-term postoperative period. Histopathological examination revealed the giant cell arteritis of the internal carotid artery. Conclusion This case emphasizes the importance of open surgical treatment of extracranial carotid artery aneurysms, which allows to perform optimal carotid artery reconstruction and also define the rare etiology of disease.

Abstract Background Giant cell arteritis (GCA) is the most common systemic vasculitis in adults. Presenting features include new-onset headaches, constitutional symptoms, jaw claudication, polymyalgia rheumatica, and visual symptoms. Arterial inflammation with subsequent stenosis and occlusion may cause tissue ischemia, leading to blindness, strokes, and myocardial infarction. Oral antiplatelet therapy has been hypothesized to reduce GCA-related ischemic events. However, previous studies have demonstrated conflicting results regarding the efficacy of antiplatelet agents in GCA. The objective of this systematic review is to assess the safety and efficacy of antiplatelet therapy for the prevention of these events in adults with giant cell arteritis. Methods In this systematic review, we will include randomized controlled trials (RTCs), quasi-randomized trials, non-randomized intervention studies, cohort studies, and case–control studies on patients with new-onset or relapsing GCA. The intervention of interest will be pre-existing use or initiation of an oral antiplatelet medication (aspirin, clopidogrel, prasugrel, or ticagrelor) at GCA onset or relapse. The comparator of interest will be the absence of antiplatelet therapy. Endpoints will be evaluated after 6 and 12 months of follow-up. The primary outcome will be GCA-related ischemic events, including permanent blindness, stroke, myocardial infarction, and ischemic event-related deaths. Adverse events such as major bleeding and death caused by a bleeding event will be assessed. Discussion GCA-related ischemic events are catastrophic, sudden, often irreversible, and lead to significant morbidity. Antiplatelet agents are affordable, accessible, and could be effective for the prevention of these events. Nevertheless, the potential benefits of platelet aggregation inhibition must be weighed against their associated risk of bleeding. Assessing the efficacy and safety of antiplatelet therapy in GCA is therefore clinically important. Systematic review registration Our systematic review protocol was registered with the International Prospective Register of Systematic Reviews (PROSPERO, registration number CRD42023441574.

The objective of this case series is to describe the efficacy and safety of baricitinib (BARI) in a group of patients with polymyalgia rheumatica (PMR) and/or giant cell arteritis (GCA). These patients were treated with BARI due to either a refractory disease course or the unavailability of tocilizumab because of the pandemic. A total of six patients (five females and one male, median age 64 years, range 50-83) were treated with BARI. Two of them had isolated PMR, two had PMR with associated large vessel (LV)-GCA, one had LV-GCA presenting as fever of unknown origin, and one had cranial-GCA. All patients reported improvement with BARI. At the time of starting BARI, patients were taking a median prednisone dose of 8.75 mg/day (range 0-25), and the four patients with PMR had a median PMR-AS of 23.3 (indicating high disease activity), which decreased to 1.58 after 6 months of treatment with BARI. Two of them could stop glucocorticoids (GC) and continued BARI monotherapy. One patient suffered from pneumonia, and BARI was therefore stopped. No other adverse events attributable to BARI were detected. Our case series supports previous reports suggesting efficacy of Janus kinase inhibitors as a GC-sparing strategy in PMR and GCA.

Key Clinical Message Giant cell arteritis (GCA) is an inflammatory vasculitis that affects larger blood vessels, like the temporal arteries and the aorta. It is systemic and tends to affect individuals over 50. Common symptoms include headache, jaw pain provoked by chewing, and fever. We present an interesting case of a GCA variant.

Large vessel vasculitis (LVV), including Takayasu's arteritis (AT, or non-specific aortoarteritis) and giant cell arteritis (GCA), is caused by granulomatous inflammation affecting mainly the aorta and its main branches. Damage to the vascular wall leads to ischemia of the corresponding organs and can be complicated by loss of vision, cerebral insufficiency and other life-threatening phenomena. The early diagnosis of these diseases in clinical practice is a difficult task that can only be solved by comparing the clinical symptoms, the results of the physical, laboratory and instrumental examination and the vascular biopsy.A comparative analysis of the 2018 and 2023 EULAR recommendations for imaging in LVV is presented. Duplex ultrasound (USDS) of not only temporal but also axillary arteries is recommended for GCA and magnetic resonance imaging (MRI) for AT. Alternative methods for GCA are MRI or positron emission tomography (PET) in combination with computed tomography (CT) and intravenous administration of fluorodeoxyglucose labelled with the short-lived fluoride isotope 18 (FDG-PET/CT), and for AT – FDG-PET/CT, CT or ultrasound examination. MRI, CT or ultrasound can be used for long-term monitoring of structural damage, especially to assess pre-existing vascular inflammation.

The Giant cell arteritis (GCA) is the most common form of systemic vasculitis in elderly patients. The treatment includes high doses of steroids and interleukin (IL)-6 inhibitor tocilizumab, especially in refractory or relapsing disease or in cases where there is an increased risk of steroid-related adverse events. This report discusses the case of a patient with giant cell arteritis who underwent treatment with tocilizumab for four years. The treatment was successful and resulted in clinical remission. However, four years after starting the therapy, the patient developed spontaneous hematomas on their extremities. After further investigation, it was discovered that the patient had developed thrombocytopenia and hypofibrinogenemia, which required substitution therapy. Malignancy and immune-mediated causes of hypofibrinogenemia and thrombocytopenia were ruled out. The patient experienced an extended period of hypofibrinogenemia that lasted for two months after the last dose of tocilizumab. During this time, the levels of CRP remained very low. This could be because the continued inhibition of IL-6 caused impaired hepatic synthesis of acute phase response proteins, which led to low fibrinogen and CRP levels in serum. The purpose of this case-based review is to emphasize the necessity of regular fibrinogen check-ups in GCA patients treated with tocilizumab.

The role of 18F-fluorodeoxyglucose (FDG) positron emission tomography (18F-FDG PET) in the diagnosis of large vessel vasculitis (LVV) is well established. It permits us to assess the extent and the grade of vascular involvement and to rule out the other causes in clinical scenarios characterized by less specific symptoms. The advantages of 18F-FDG PET are far less clear in monitoring disease activity over time. Studies looking for the role of 18F-FDG PET as a potential biomarker had conflicting results and whether and when to repeat it during follow-up is based on clinical experience. A comprehensive assessment, including clinical, laboratory and morphological imaging is still required to monitor patients with large-vessel vasculitis over time. The aim of this review is to present more recent data about the utility of 18 F-FDG PET in the diagnosis and follow-up of LVV.

Giant cell arteritis (GCA) may manifest with aggressive intracranial stenosis resistant to medical therapy, and patients may develop refractory neurologic deficits and cerebral infarcts, making GCA a life-threatening condition. We report the case of a 68-year-old woman recently diagnosed with GCA, medicated with prednisolone 60 mg daily. Two weeks later, the patient was admitted to our Stroke Unit after a sudden episode of global aphasia. Magnetic resonance angiography showed two recent ischaemic lesions, besides an erythrocyte sedimentation rate of 17 mm/hour. A cerebral angiography revealed bilateral stenosis and dilation in the petrous, cavernous and supraclinoid segments of internal carotid arteries (ICA). The patient was started on intravenous methylprednisolone pulses (250 mg daily for five days). Computed tomography (CT) angiography and Doppler ultrasound showed severe vascular disease affecting multiple territories, without significant intracranial involvement. The hypothesis of GCA with extracranial vasculitic involvement was considered as the aetiology of ischaemic cerebral infarctions in multiple territories and, given the severity of the disease, it was decided to add tocilizumab. Despite this, the patient evolved with significant worsening neurological deficits and a CT scan confirmed the presence of new vascular events. Endovascular treatment (EVT) with balloon angioplasty was conducted on both ICAs, with improved calibre and downstream filling. After that, the patient presented sustained clinical improvement, without recurrence of any ischaemic events at the one-year follow-up. This clinical case stands out for the importance of EVT as an effective therapy in patients with medically refractory GCA with symptomatic intracranial stenosis, improving their prognosis.

IntroductionTemporal artery (TA) biopsy is commonly used for the diagnosis of giant cell arteritis (GCA). However, a positive biopsy is no longer mandatory for diagnosis. This study aims to correlate the histopathological findings of TA biopsies in suspected cases of GCA to the clinical presentation in an ophthalmic tertiary eye care center to draw useful conclusions and advocate the possible implementation of guidelines for TA biopsy.MethodsData was collected from patients’ medical records including, demographics, clinical data, and histopathological findings and diagnosis. The 2022 American College of Rheumatology/ European Alliance of Associations for Rheumatology (ACR/EULAR) criteria have been used and partially adopted as a guide to compare the variables between TA biopsy-positive and negative groups as well as the TA biopsy-positive group and the group of patients with TA biopsy showing atherosclerosis.ResultsOut of the total 35 patients who underwent a TA biopsy during the period of 23 years, 22.9% of patients had histopathological findings consistent with GCA and 42.9% had TA atherosclerotic changes, while the remaining 34.3% had histologically unremarkable TA. The mean age of all patients was 66 ± 10.9 years. Slightly more than half were females (54.3%) and the remaining were males (45.7%). In the group with positive TA biopsies, the mean age was 71 ± 8.4 years with a higher female predominance (female-to-male ratio of 5:3). The mean diagnostic clinical score used in our study was higher (7.5 ± 2.33) in the GCA-positive group when compared to the other groups with statistical significance (mean of 4.85 ± 2.01 in patients with overall GCA-negative biopsies and 5.13 ± 2.10 in the group with atherosclerosis). Other three clinical variables that were found to be statistically significant in the GCA biopsy-positive group were scalp tenderness, jaw claudication, and optic nerve pallor.DiscussionThe mean age (71 ± 8.4 years) and the female predominance of GCA in our group of patients with positive TA biopsy (62.5%) was like other reports. In our study 22.9% of performed TA biopsies over the period of the study were positive confirming the diagnosis of GCA on histological exam, which was similar to another report and is considered to be relatively low. The incorporation of increased clinically focused assessments and algorithms, with the aid of the ACR/EULAR criteria, may decrease the frequency of TA biopsies that carries unnecessary cost and risk of procedure-related morbidity. We highly recommend applying the age of ≥ 50 years as an initial criterion for diagnosis, followed by the consideration of the statistically significant clinical features: scalp tenderness, jaw claudication, and optic nerve pallor.

Abstract Background Polymyalgia rheumatica and giant cell arteritis are systemic inflammatory conditions of the elderly. Polymyalgia rheumatica classically presents as a bilateral proximal muscle pain and stiffness syndrome. Biceps tenosynovitis is the commonest pathology in polymyalgia rheumatica. However according to literature, erosive sternoclavicular arthritis is a rare association of polymyalgia rheumatica. Giant cell arteritis is an inflammatory granulomatous arteritis predominantly involving large cerebral arteries. Thus, its classic clinical presentation includes severe headache with scalp tenderness, jaw claudication, and sudden painless loss of vision. Urological manifestations (prostatic vasculitis and epididymo-orchitis) were seldom reported in giant cell arteritis. Case presentation A 53-year-old Sinhalese man presented with progressive right-sided shoulder joint pain and neck pain associated with constitutional symptoms and episodic generalized headache. Examination revealed restricted movements of the right shoulder joint with nontender pulsatile bilateral temporal arteries. Blood testing showed elevated erythrocyte sedimentation rate and C-reactive protein. Color Doppler ultrasound of the superficial temporal artery revealed “halo sign.” The temporal artery showed infiltration of mononuclear cells in the arterial media and adventitia. Computed tomography revealed right sternoclavicular arthritis with incidental finding of ureteric stricture. The patient was treated with high-dose oral prednisolone, and good clinical and biochemical response was observed during follow-up. Conclusion Polymyalgia rheumatica–giant cell arteritis may rarely present as erosive sternoclavicular arthritis as the initial manifestation, mimicking many rheumatological conditions. Urological involvement such as ureteric strictures may be rare associations of primary systemic vasculitis. A high degree of suspicion combined with targeted investigations would allow early identification the polymyalgia rheumatica–giant cell arteritis syndrome in the presence of atypical manifestations, leading to improved patient outcomes.

Abstract Background Giant cell arteritis is a large vessel vasculitis of the arteries in the head and neck. The mainstay of management is with high-dose corticosteroids, and patients often face difficulties stopping or reducing steroids without recurrence of symptoms. Corticosteroids are well established to have numerous associated side effects, including osteoporosis, weight gain, and diabetes. Therefore, when tocilizumab was approved for up to 1 year for cases of relapsing or refractory giant cell arteritis by the National Institute of Health and Care Excellence (NICE) in April 2018, this offered an opportunity to benefit from new funding and to reduce steroid burden. Case presentation This case series describes the impact of the establishment of a new hub and spoke referral pathway for the use of tocilizumab in refractory or relapsing giant cell arteritis, with case examples from consecutive patients who accessed the funding between August 2018 and April 2021. A total of 16 patients were identified: 11 female and 5 male, with an average age of 72.4 (range 61–82) years, with a majority of 11 ethnically white. The central assessing hub is St George’s University Hospitals NHS Foundation Trust Hospital, serving a population of 1.3 million in the south of England. This is the first large case series looking into the impact of the establishment of a regional clinical pathway for the new tocilizumab funding. Conclusions The case series demonstrates that the use of tocilizumab has reduced both the duration and the dose of corticosteroids in these 16 cases (mean prednisolone reduction 20.4 mg: 95% CI 13.0–27.8 mg), with 50% of patients continuing on tocilizumab after the initial 12 month funding period. The disease course, patterns of response, and maintenance of remission are discussed, and we describe the benefits of replicating this hub and spoke tocilizumab pathway in other centers.

Key Clinical Message Prompt diagnosis and treatment of GCA are crucial to preserve vision. Because of this, new‐onset ptosis or diplopia in elderly patients should warrant consideration of GCA, even in the absence of “classic” features, systemic symptoms or elevated inflammatory markers. Abstract Giant cell arteritis (GCA) is a vision‐threatening, ophthalmic emergency that classically presents with new‐onset headaches, scalp tenderness, systemic symptoms, visual disturbances, and elevated inflammatory markers. We describe an atypical presentation of GCA in an 87‐year‐old patient with fatigable ptosis and diplopia, with subsequent anterior ischemic optic neuropathy and choroidal infarction.

This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.

Systemic vasculitis is a manifold group of systemic autoimmune diseases characterized by the inflammation of the blood vessels. The first clinical cases of systemic vasculitis were described in the Middle Ages, and most of the currently recognised nosological forms were reported in the first half of the 20th century. The first attempt to create a united classification of vasculitis was performed by P. Zeek in 1952. In the following decades accumulation of the data on the etiology and pathogenesis of different vasculitis guided researchers from different countries in their attempts to improve classification. The main principles of classification were the size of the affected blood vessels, disease etiology and pathogenesis. In 1990 American College of Rheumatology (ACR) published classification criteria for seven forms of the systemic vasculitis, that gave a significant contribution to the conduction of large-scale studies in this field. However, the first international nomenclature of vasculitis was developed only in 1994 during the Consensus Conference in Chapel Hill. Revised and augmented version of this nomenclature was created in 2012 and is still valid. An important step in the development of the classification of vasculitis was a joint project of ACR and EULAR aimed to develop new diagnostic and classification criteria for vasculitis (DCVAS). The first result of this project are the new classification criteria for granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis published in 2022. In general, the evolution of the classification of vasculitis occurs under the influence of the progress in the understanding of their etiology and pathogenesis.

Giant cell arteritis (GCA) is the most prevalent subtype of vasculitis in adults. In recent years, there has been substantial improvement in the diagnosis and treatment of GCA, mainly attributed to the introduction of highly sensitive diagnostic tools, incorporation of modern imaging modalities for diagnosis and monitoring of large-vessel vasculitis, and introduction of highly effective novel biological therapies that have revolutionized the field of GCA. This article reviews state-of-the-art approaches for the diagnosis, monitoring, and treatment options of GCA.

Abstract Clinical presentation as well as histological or biological findings can sometimes make the diagnosis of giant cell arteritis difficult. Histopathological features of temporal artery biopsy from giant cell arteritis patients are also challenging because of the various described appearances or even finding of clinically normal temporal artery biopsy does not rule out the diagnosis. We here describe the case of a 51‐year‐old man with temporal artery biopsy showing lymphocytes infiltrates in the adventitia corresponding to the so‐called adventitial pattern of giant cell arteritis according to Hernandez‐Rodriguez et al.

Abstract Background Giant cell arteritis has a wide variety of clinical symptoms, one of them being cervical radiculopathy, which mainly involves the C5 nerve root. If the patient does not develop typical clinical symptoms of giant cell arteritis but has C5 radiculopathy, it may be misdiagnosed as polymyalgia rheumatica or elderly-onset rheumatoid arthritis due to old age, high serum inflammatory markers, and difficulty in raising both upper limbs. Case presentation A 72-year-old Japanese man with a month-long history of dyspnea on exertion and with difficulty in raising both upper limbs was referred to our hospital because of elevated serum C-reactive protein (12.62 mg/dL). He had no typical symptoms of giant cell arteritis such as headache, jaw claudication, visual loss, and fever. The patient tested negative for rheumatoid factor and anti-cyclic citrullinated peptide antibody, and matrix metalloproteinase-3 was within the normal range (54.3 ng/mL). Musculoskeletal ultrasound examination showed absence of tenosynovitis, bursitis, and synovitis, and the patient did not meet the classification criteria of polymyalgia rheumatica or rheumatoid arthritis; hence, those two diseases were unlikely. A precise neurological examination suggested bilateral C5 and C6 anterior radiculopathy and left C4 radiculopathy. Since cervical magnetic resonance imaging showed no mechanical causality, cervical radiculopathy of unknown origin was suggested. Fluorodeoxyglucose positron emission tomography/computed tomography revealed increased fluorodeoxyglucose lineal uptake along the vessel walls, including temporal arteries, vertebral arteries, and axillary arteries. Results of the biopsy of the left superficial temporal artery were compatible with giant cell arteritis. He was successfully treated with 30 mg of prednisolone, and both upper limbs could be elevated. Conclusions If the patient was misdiagnosed with polymyalgia rheumatica or elderly-onset rheumatoid arthritis based on only clinical symptoms and laboratory data, his symptoms might not improve due to insufficient steroid dose and vascular complications may occur later. Although rare, peripheral neuropathy in giant cell arteritis may include cervical radiculopathy. The musculoskeletal ultrasound and precise neurological examination were the turning points for the diagnosis of this case, and making a careful diagnosis using these methods was important.

Temporal arteritis (TA) is an inflammatory vascular disease common in the European population. It is mainly characterized by sudden onset headache. TA is rarely associated with other autoimmune diseases, such as Sjögren’s syndrome (SS). We present the case of a Peruvian 71 year-old man with SS history, who was admitted to the emergency department due to severe headache evolved in 4 days, periocular pain and right ptosis. The authors also performed a systematic review of case reports or case series of patients diagnosed with both TA and SS. This temporal arteritis case is an atypical presentation because headache was characterized by mixed nociceptive and neuropathic pain components. Despite the infrequency, new studies should be carried out to identify comorbidities in TA patients.

Abstract Background Given the absence of consensus diagnostic criteria for giant cell arteritis, clinicians may encounter difficulty with identification of new-onset headache in patients older than age 50 years presenting with visual changes and elevated inflammatory markers, particularly if temporal artery biopsies are performed and negative. Case presentation We present a case of a 57-year-old white man with headache, diplopia, and jaw paresthesia initially diagnosed and managed as steroid-refractory biopsy-negative giant cell arteritis. Further investigation disclosed evidence of soft tissue infiltration into Meckel’s (trigeminal) cave bilaterally. Positron emission tomography suggested the presence of a lymphoproliferative disorder. Histology confirmed the diagnosis of diffuse large B cell lymphoma. Conclusions Metastatic involvement in Meckel’s cave in diffuse large B cell lymphoma is extremely rare and presents a diagnostic challenge. Patients with suspicion of giant cell arteritis should undergo advanced imaging, particularly those with negative biopsy, atypical features, or lack of response to standard therapy, in order to assess for the presence of large-vessel vasculitis or other mimicking pathologies.

Abstract Background and aim Giant cell arteritis (GCA) remains a medical emergency because of the risk of sudden irreversible sight loss and rarely stroke along with other complications. Because headache is one of the cardinal symptoms of cranial GCA, neurologists need to be up to date with the advances in investigation and management of this condition. The aim of this document by the European Headache Federation (EHF) is to provide an evidence-based and expert-based recommendations on GCA. Methods The working group identified relevant questions, performed systematic literature review and assessed the quality of available evidence, and wrote recommendations. Where there was not a high level of evidence, the multidisciplinary (neurology, ophthalmology and rheumatology) group recommended best practice based on their clinical experience. Results Across Europe, fast track pathways and the utility of advanced imaging techniques are helping to reduce diagnostic delay and uncertainty, with improved clinical outcomes for patients. GCA is treated with high dose glucocorticoids (GC) as a first line agent however long-term GC toxicity is one of the key concerns for clinicians and patients. The first phase 2 and phase 3 randomised controlled trials of Tocilizumab, an IL-6 receptor antagonist, have been published. It is now been approved as the first ever licensed drug to be used in GCA. Conclusion The present article will outline recent advances made in the diagnosis and management of GCA.

Objective: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations. Methods: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients’ representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations. Results: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids. Conclusions: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are both rheumatological diseases of the elderly with a strong association with each other and which rarely present with normal inflammatory markers. Here we present the case of a 61-year-old Caucasian woman who had typical symptoms of both diseases. At the time of presentation, her blood work showed normal inflammatory markers, but because of the high clinical suspicion for GCA, a temporal artery biopsy was done which was positive for giant cells and disruption of the internal elastic lamina. Our patient responded very well to treatment with oral steroids and steroid-sparing medication and was able to return to her normal life without experiencing any complications of the disease. By sharing our case, we aim to increase the awareness of medical personnel regarding the importance of focusing on the clinical presentation as well as the laboratory and pathological aspects of diagnosing GCA and PMR.

Giant cell arteritis is the most common type of systemic vasculitis. An increased risk of venous thromboembolism has been described in these patients. We report the case of a 79-year-old woman with a history of polymyalgia rheumatica, who presented with left thoracic pain radiating to the neck and scapula plus temporal headache. She had no changes on physical examination, but work-up tests showed increased D-dimer levels and computed tomography pulmonary angiography revealed signs of a chronic/subacute embolism in the right inferior lobe. Anticoagulation with edoxaban was started after 5-day bridging with enoxaparin. Three weeks after the initial diagnosis the headache still persisted and she developed scalp tenderness. Giant cell arteritis was diagnosed and treated with prednisolone, with complete resolution of symptoms. Extensive diagnostic work-up was performed to identify an alternative cause of pulmonary thromboembolism; however, the investigations were negative. This case supports the hypothesis that this type of vasculitis could be related to the occurrence of pulmonary embolism.

We report a 78-year-old man presenting with persistent headaches in vertex and temporo-parietal area; fatigue, worsening after walking; jaw claudication; scotomas; pharyngodynia; and dry cough after the second dose of the SARS-CoV-2 vaccine (ChAdOx1-S) administration. Laboratory findings showed an elevated C-reactive protein level and FDG-CT PET showed evidence of active large vessel vasculitis with diffuse abnormal artery uptake. Under suspicion of vasculitis, a temporal arteries biopsy was performed; the histopathologic findings demonstrated the transmural inflammatory infiltrate with giant cells, compatible with giant cell arteritis. Although the overall incidence of vaccine-triggered autoimmunity is low, rheumatologists worldwide should be aware of autoimmune diseases as a new potential adverse event of vaccines.

Abstract Here, we describe a case of giant cell arteritis (GCA) simultaneously diagnosed with chronic subdural hematoma. In this case, head to chest computed tomography angiography was useful for the diagnosis and treatment of GCA.

Abstract GCA is not always a linear diagnosis. Rarely reported as a paraneoplastic condition when associated with solid tumors, the available cases are associated with poor response to corticosteroids.

Giant cell arteritis is a medical emergency as severe, irreversible complications may occur if it is not treated in a timely manner. However, in daily practice early diagnosis can be challenging. We report the case of a 70-year-old woman who presented with multiple ischaemic cerebral vascular accidents related to newly diagnosed giant cell arteritis. Review of her charts revealed a substantial delay from the onset of symptoms to diagnosis. This case demonstrates the need for additional efforts to reduce delay in referring patients with giant cell arteritis and the need to implement fast-track clinics to prevent serious complications.

AIMS To describe the feasibility and diagnostic accuracy of 18F-FDG positron emission tomography-computed tomography (PET/CT) of the temporal artery compared with temporal artery ultrasound and histology of the temporal artery in patients with suspicion of having giant cell arteritis (GCA). MATERIALS AND METHODS Patients with suspected GCA were included. PET/CT standard uptake value ratios and the compression sign on ultrasound were assessed for the trunk, and parietal and frontal branches of the temporal artery. Temporal artery biopsies were systematically re-assessed, if available. RESULTS In 17/34 patients, GCA was confirmed. Temporal artery PET/CT confirmed vasculitis in 9/17 patients and was negative in all 17 controls. Nineteen of 34 subjects had a temporal artery biopsy, which was positive in 7 patients. Five of these seven were negative in the preceding PET/CT. Ultrasound confirmed vasculitis in 9/17 patients and was negative in 16/17 controls. In 7/17 patients, PET/CT and ultrasound were positive for temporal arteritis. Two patients had positive findings only on temporal artery PET/CT and two patients showed vasculitis only on temporal artery ultrasound. No temporal artery segments

Abstract Introduction Temporal artery biopsy is essential for the diagnosis of giant cell arteritis. It has been shown that 18F-fluorodeoxyglucose positron emission tomography-computed tomography, magnetic resonance angiography, and ultrasonography are useful for the diagnosis of giant cell arteritis. However, there are only a few reports on the usefulness of three-dimensional computed tomography angiography in the diagnosis of giant cell arteritis. We describe two cases in which giant cell arteritis was difficult to diagnose using positron emission tomography-computed tomography and magnetic resonance angiography but was diagnosed using three-dimensional computed tomography angiography, thus showing the importance of three-dimensional computed tomography angiography in the diagnosis of giant cell arteritis. Case presentation Case 1: An 81-year-old Japanese man. Laboratory investigations revealed normocytic anemia and raised inflammatory marker levels. Slight bleeding in the right posterior pole of his eyeball and leukoma of his left cornea were observed on fundus examination. Stenosis and stoppage of the temporal artery were detected on three-dimensional computed tomography angiography. A diagnosis of giant cell arteritis was made, and he was started on orally administered prednisolone. His headache and C-reactive protein levels improved. Four weeks after glucocorticoid steroid treatment, three-dimensional computed tomography angiography revealed improvement in stenosis and stoppage of temporal artery. Case 2: A 74-year-old Japanese woman. A dose of 20 mg of prednisolone was administered and her polymyalgia and polyarthritis improved; however, her headache and ear occlusion persisted. Although vasculitis was not detected on positron emission tomography-computed tomography, stenosis and stoppage of the temporal artery were detected on computed tomography angiography. She was diagnosed as having giant cell arteritis and started on orally administered prednisolone treatment (60 mg daily). Her headache and C-reactive protein levels improved. Four weeks after glucocorticoid treatment, three-dimensional computed tomography angiography showed improvement in stenosis and stoppage of temporal artery. Conclusions In both patients with giant cell arteritis, three-dimensional computed tomography angiography revealed improvement in stenosis and stoppage of temporal artery after glucocorticoid treatment. We conclude that computed tomography angiography along with magnetic resonance angiography, positron emission tomography-computed tomography, and ultrasonography are important for the diagnosis of giant cell arteritis.

Stroke is a severe medical condition, which should be diagnosed ant treated rapidly. During imaging diagnostics atypical appearance of vessels may suggest other than thrombotic occlusion. Circumferential vascular stenosis is typical for giant cell arteritis, which should be considered as a differential diagnosis and treated with corticosteroids.

Introduction: Giant Cell arteritis can be difficult to diagnose clinically. In those cases, where there is no certainty, there is more reliance on a temporal artery biopsy and radiological imaging to confirm the diagnosis. The purpose of this article was to identify the standard of care in individuals with suspected Giant Cell Arteritis in a typical district general hospital and to offer a proposed pathway for treatment. Methods: Darent Valley Hospital has been managing Giant Cell Arteritis for many years but there has always been a need for an outlined pathway to identify those at risk of cranial complications like visual loss to improve patient care. We evaluated the management of 70 individuals that had a temporal artery biopsy and followed their treatment journey. We extracted clinical specialist, emergency admission, operation theatre and histological data. We collected clinic follow up data over the following years to identify those that relapsed on treatment, stayed in remission or had complications. We propose a pathway to manage those individuals with Giant Cell arteritis in line with the new advances in treatment. Results: Ten patients were identified that had a histologically positive biopsy. Reassuringly, most individuals with an obvious clinical diagnosis had high dose glucocorticoid treatment commenced before even being referred for a biopsy. Nine individuals had visual ischemia out of which five lost their vision. Conclusion: The presentation of a pathway will help streamline best medical and surgical practice and ensure the availability of urgent specialist treatment and to identify those at risk of ischemic complications.

Background and objectives: Although a lot of studies suggested the predisposition of many risk factors, non-arteritic anterior ischemic optic neuropathy was considered for a long time to be idiopathic. This study aimed to assess the relationship between different factors and anterior ischemic optic neuropathy among a sample of patients in Erbil city. Methods: A case-control study was conducted in Erbil city, Iraq from 2008 to 2011. A total of 36 cases of non-arteritic anterior ischemic optic neuropathy and 36 controls with other visual problems were included. Results: Eight percent of patients in this study had one or more risk factors (P = 0.001), in particular hypertension and diabetes were shown to have a significant relationship (P = 0.046 and 0.001, respectively). The absence of physiological cup over the optic disc has crucial importance both independently (P = 0.001) and in association with diabetes (P = 0.001). Old age and male gender were significant factors in relation to co-morbidities (P = 0.001 and 0.038, respectively). Conclusion: The study had concluded that hypertension, diabetes, age, male gender and absent physiological cup over the optic disc were important risk factors for anterior ischemic optic neuropathy.

Giant cell arteritis (GCA) is the most common form of vasculitis of the adult. General practitioners (GPs) are usually the first physicians who take care of GCA patients. In this study, the awareness of GPs from Genoa, Italy, regarding GCA was investigated by a web-based survey. A web-based questionnaire was sent by mail to 775 Italian GPs. It included 12 multiple choice questions regarding practice seniority, practice population size, number of GCA patients followed, and GPs’ diagnostic and therapeutic approach. Of the 775 GPs involved, 76 (9.8%) answered. Thirty-three/75 (44%) declared that they did not see patients with GCA and the remaining 42 (56%) that they diagnose between one and two patients per year. New headache onset was the presenting feature of GCA for the majority of GPs (78.3%). GCA was diagnosed on the basis of clinical presentation alone by 35.2% of them, of temporal artery biopsy by 49.3%, and by imaging, including ultrasound and magnetic resonance imaging, by 15.5%. The referral pattern was mainly toward rheumatologists (68.5%). Only 27.8% GPs declared they start treatment at the first clinical suspicion, with the others waiting for laboratory and imaging examinations or specialist consultation. The doses of glucocorticoids used were in keeping with current guidelines. The management of GCA by GPs from Genoa is in general correct, with the exceptions of excessive confidence in headaches for diagnosis and of the timing of GC initiation. These points suggest that a program of information and education for GPs is warranted.

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown etiology, commonly occurring in the elderly and is associated with a good prognosis. Patients usually present with pain in the neck, shoulders, and hips. The onset is often abrupt and is associated with depression and flu-like symptoms. Lung involvement in patients with PMR is unusual. Here we report a rare case of a 66-year-old man who presented with clinical features of PMR and respiratory symptoms, namely exertional dyspnea and dry cough.

Autoimmune diseases (AID) have been associated with a variety of lymphoproliferative disorders. Multiple myeloma (MM), one of the most common haematologic malignancies characterized by clonal proliferation of bone marrow plasma cells, has been associated with a range of autoimmune disorders. In this report, we described a case study of a patient admitted to our Internal Medicine Department for a bone marrow biopsy and myelogram due to a monoclonal peak observed by his general practitioner. However, at admission he presented typical giant cell arteritis (GCA) complaints, suggesting the coexistence of both diseases. The possible pathogenesis, as found in the literature, explaining the association will be discussed.

Introduction: The American College of Rheumatology (ACR) criteria, and more recently the revised ACR criteria (rACR), are a scoring system developed to aid in the diagnosis of giant cell arteritis (GCA). Our aim was to investigate the value of the non-biopsy criteria of the original ACR criteria and rACR criteria to predict GCA, and investigate the utilization of such scores to avoid biopsy when a very high or very low likelihood of a positive temporal artery biopsy TAB was predicted. Method: We conducted a retrospective cohort study of 59 patients undergoing TAB from 2013 to 2017 in Beaumont Hospital, a tertiary referral centre in Dublin, Ireland. Demographic data, biochemical results, presenting features, and histology results were collected and collated. Results: Data were analysed from 53 patients and ACR scores were compiled. Seventeen scored < 3 and thirty-six scored 3–5. All 11 positive biopsies were in the 3–5 score range. Forty-five patients were analysed with rACR scores. Eight were excluded due to not meeting the inclusion criteria. Of the 11 positive biopsies, 2 were in the 3–4 score range, and 9 were in the ≥5 score range. In the ACR method, 36% of all biopsies scored as low-risk pre-biopsy. In the rACR method, 84.4% of all biopsies scored in the low- and intermediate-risk group pre-biopsy and 15.6% of all biopsies scored in the high-risk group pre-biopsy. Conclusions: This study illustrates the potential value of the rACR scoring system as a useful tool to categorize patients according to risk with a view to avoiding unnecessary TAB. The data suggest that a TAB has a helpful role in low- and intermediate-risk groups but is of minimal benefit in the high-risk group.

Abstract Background Glucocorticoids are associated with increased fracture risk and are the mainstay of treatment in polymyalgia rheumatica (PMR) and giant cell arteritis (GCA). However, fracture risk in these conditions has not been previously quantified. The aim of this study was to quantify the risk of fracture among patients with PMR and GCA. Methods A retrospective cohort study was conducted using primary care records from the UK-based Clinical Practice Research Datalink. Individuals aged 40 years and over, with incident diagnoses of PMR or GCA were separately identified from 1990–2004 and followed up until 2015. For each exposed individual, four age-, sex- and practice-matched controls were randomly selected. Incidence rates of fracture per 10,000 person-years were calculated for each disease group and hazard rates were compared to the unexposed using Cox regression models. Results Overall, 12,136 and 2673 cases of PMR and GCA, respectively, were identified. The incidence rate of fracture was 148.05 (95% CI 141.16–155.28) in PMR and 147.15 (132.91–162.91) in GCA per 10,000 person-years. Risk of fracture was increased by 63% in PMR (adjusted hazard ratio 1.63, 95% CI 1.54–1.73) and 67% in GCA (1.67, 1.49–1.88) compared to the control populations. Fewer than 13% of glucocorticoid-treated cases were prescribed bisphosphonates. Conclusions This study reports, for the first time, a similar increase in fracture risk for patients with PMR and GCA. More needs to be done to improve adherence to guidelines to co-prescribe bisphosphonates. Further research needs to identify whether lower glucocorticoid starting doses and/or aggressive dose reduction reduces fracture risk.

Nowadays, there is no doubt about the relationship between immune inflammation and the development of thrombosis due to the similarity of many pathogenetic machanisms underlying both pathological processes. Systemic vasculitis, along with other immuno- mediated inflammatory diseases, represent one of the most clear examples of such interaction. Thrombotic complications remain one of the most serious and life-threatening conditions that occur in patients with autoimmune diseases, in particular systemic vasculitis. This serves as powerful stimulus for studing the problem of hypercoagulation, which often accompanies the course of systemic vasculitis. Of interest are thrombosis that occure both in the injury of large vessel and in the involvement of medium and small vessels in the pathological process. According to the literature, thrombotic and thromboembolic complications are most common in ANCA-associated vasculitis and Behçet’s disease. This review discusses current studies regarding features of clinical picture and mechanisms of thrombosis development in systemic vasculitis. Because of these studies it became clear that for some vasculitis a high frequency of both arterial and venous thrombosis is characteristic. At the same time, other vasculitis are accompanied by high risk of only venous thrombosis. Finally, thrombosis and thromboembolism are quite rare complications for some vasculitis. Among the presented mechanisms of thrombosis involvement, disruptions of the hemostasis are widely considered. For examples, the presents the results of researches, which is actively discussed the role of neutrophil extracellular traps and antiendothelial cell antibodies in the development on thrombotic complictions. Special attention is paid to the possible role of some disorders of hemostasis, such as polymorphism V coagulation factor G1691FA and prothrombin G20210A. To search for literature sources, the following queries were used: “vasculitis”, “thrombosis”, “neutrophil extracellular traps”, “ANCA-associated vasculitis”, “venous thrombosis”, “arterial thrombosis”, “Behçet’s disease”, “Takayasu arteritis”, “granulomatosis with polyangiitis”, “eosinophilic granulomatosis with polyangiitis”, “Mmicroscopic polyangiitis”, “Henoch–Schönlein purpura”, “Kawasaki disease”, “polyarteritis nodosa”, “giant cell arteritis”, “Buerger’s disease”. In addition, use similar requests in English. The search was carried out in PubMed.

Giant cell arteritis (GCA) is an inflammatory disease involving mainly the cranial arteries branches. We present a case of an old male with right hemiparesis that was admitted to our hospital. The subject’s daughter stated that the patient had gone to another clinic 3 months ago because he experienced anorexia and temple headache. A temporal artery biopsy proved GCA. Prednisone was started. Within one month, the prednisone-dose was 40mg/day. The patient was asymptomatic, but the prednisone-dose was maintained because he had elevated inflammatory markers. On admission, the subject had right limbs weakness and global aphasia with six hours of onset. Twelve hours after admission, the patient developed a seizure, and a CT-scan showed a hypodense area in the territory of the left middle cerebral artery. Upon further questioning, the subject’s family admitted that he reduced the dose of prednisone of 40 mg/day to 10-20 mg/day without any medical advice 2-weeks after the stroke.

The paper describes clinical cases in 2 patients (brothers) with giant cell arteritis. It analyzes the genetic and epigenetic aspects of the disease. The data available in the Russian and foreign literature are given.

Abstract Background Giant cell arteritis (GCA), if untreated, can lead to blindness and stroke. The study’s objectives were to (1) determine a new evidence-based benchmark of the extent of diagnostic delay for GCA and (2) examine the role of GCA-specific characteristics on diagnostic delay. Methods Medical literature databases were searched from inception to November 2015. Articles were included if reporting a time-period of diagnostic delay between onset of GCA symptoms and diagnosis. Two reviewers assessed the quality of the final articles and extracted data from these. Random-effects meta-analysis was used to pool the mean time-period (95% confidence interval (CI)) between GCA symptom onset and diagnosis, and the delay observed for GCA-specific characteristics. Heterogeneity was assessed by I 2 and by 95% prediction interval (PI). Results Of 4128 articles initially identified, 16 provided data for meta-analysis. Mean diagnostic delay was 9.0 weeks (95% CI, 6.5 to 11.4) between symptom onset and GCA diagnosis (I 2 = 96.0%; P